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Dermatomyositis | Johns Hopkins Medicine

What is dermatomyositis?

Dermatomyositis is a rare disease that causes muscle inflammation and skin rash. It’s one of a group of muscle diseases that cause muscle inflammation and swelling. It’s different from other muscle diseases because it also causes skin problems. Dermatoyositis is the term used to describe both muscle and skin symptoms.

It can occur at any age, but it most often affects adults ages 50 to 70. Women are twice as likely as men to be diagnosed with the disease. Some people with the disease also have a connective tissue disorder, such as lupus or rheumatoid arthritis.

What causes dermatomyositis?

The exact cause is not known, but possible causes include:

  • Abnormal genes you are born with

  • Cancer, especially in older people

  • Autoimmune disease, a type of illness that causes the body’s immune system to attack its own tissues

  • An infection, medication, or another exposure in your environment that triggers the disease

What are the symptoms of dermatomyositis?

The symptoms are caused by swelling and inflammation in the blood vessels that supply your skin and muscles, and can include:

  • Red or purple rash on sun-exposed areas that may be painful or itchy

  • Red or purple swelling of the upper eyelids (heliotrope)

  • Red or purple spots on the knuckles, elbows, knees, and toes (Gottron’s papules)

  • Joints that feel stuff and turn pale and painful in cold conditions and feel better when warmed (Raynaud’s phenomenon)

  • Scaly, rough, dry skin, which can lead to hair thinning

  • Swollen, red areas around the fingernails

  • Hard lumps under the skin caused by calcium deposits (calcinosis)

  • Muscle weakness in the neck, hip, back, and shoulders

  • Trouble swallowing and voice changes

  • Tiredness, fever, and weight loss

  • Muscle aches

  • Trouble rising from a chair or getting out of bed due to muscle weakness

Sometimes the muscle weakness also spreads to the heart, GI tract, and lungs. This can cause breathing trouble and coughing. Adults may have a low-grade fever, along with lung inflammation and sensitivity to light.

How is dermatomyositis diagnosed?

The process starts with a person’s medical history and a physical exam. The health care provider will look for an underlying disease, such as cancer. Tests may also be done, such as:

  • Blood tests. These are done to look for signs of muscle inflammation. They also check for abnormal proteins that form in autoimmune disease. The most common blood tests include muscle enzyme creatine kinase and the antinuclear antibody.

  • Electromyelogram (EMG). This may be done to find abnormal electrical activity in affected muscles.

  • MRI. This test uses large magnets and a computer to look for inflammation in the body.

  • Skin or muscle biopsy. Tiny pieces of tissue are taken to be checked with a microscope.

How is dermatomyositis treated?

Treatment will depend on your symptoms, your age, and your general health. There’s no cure for the condition, but the symptoms can be managed. You may need more than one kind of treatment. And your treatment may need to be changed over time. Treatments include:

  • Physical therapy. Special exercises help to stretch and strengthen the muscles. Orthotics or assistive devices may be used.

  • Skin treatment. You may need to avoid sun exposure and wear sunscreen to help prevent skin rashes. Your health care provider can treat itchy skin rashes with antihistamine drugs or with anti-inflammatory steroid creams that are applied to the skin.

  • Anti-inflammatory medications. These are steroid drugs, or corticosteroids. They ease inflammation in the body. They may be given by mouth or through an IV.

  • Immunosuppressive drugs. These are drugs that block or slow down your body’s immune system. These include the drugs azathioprine, methotrexate, cyclosporine A, cyclophosphamide, and tacrolimus.

  • Immunoglobulin. If you have not responded to other treatments, these drugs may be given. They are donated blood products that may boost your body’s immune system. They are put directly into your bloodstream through an IV.

  • Surgery. Surgery may be done to remove the calcium deposits (calcinosis) under the skin if they become painful or infected.

Talk with your health care providers about the risks, benefits, and possible side effects of all medications.

Dermatomyositis in Children

What is dermatomyositis?

Childhood dermatomyositis describes a group of muscle diseases characterized by chronic muscle inflammation followed by progressive muscle weakness.

Symptoms

Diagnosis

Careful clinical evaluation is required to determine the cause of the symptoms.

Treatment

There is no cure for dermatomyositis, but you can treat the symptoms with medication, physical therapy, exercise, heat therapy and rest. Medications include corticosteroids, immunosuppressant drugs and topical ointments.

What are the complications of dermatomyositis?

Possible complications for some people with dermatomyositis include lung disease, heart disease, or cancer. These can make treatment more difficult.

Living with dermatomyositis

If you have dermatomyositis, you may need to be treated for the rest of your life. It’s important to learn as much as you can about the disease. Work closely with your health care provider. Researchers are studying causes and treatments for the condition. Early diagnosis and treatment for the disease may improve over time.

When should I call my health care provider?

If your symptoms get worse or you notice new symptoms, notify your health care provider.

Key points

  • Dermatomyositis is a rare disease that causes muscle weakness and skin rash.

  • Symptoms include a red or purple rash on sun exposed skin and eyelids, calcium deposits under the skin, muscle weakness, and trouble talking or swallowing.

  • There is no cure, but treatment is done to reduce the symptoms.

  • Complications include lung disease, heart disease, and cancer.

Next steps

Tips to help you get the most from a visit to your health care provider:

  • Before your visit, write down questions you want answered.

  • Bring someone with you to help you ask questions and remember what your provider tells you.

  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.

  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.

  • Know how you can contact your provider if you have questions.

Autoimmune diseases in dermatology | DermNet NZ

Author: Dr Rosie Chellet, Medical Registrar, Christchurch Hospital, Christchurch, New Zealand. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, New Zealand. Copy edited by Gus Mitchell/Maria McGivern. April 2019.


What is the immune system?

The immune system is made up of the cells, molecules, and structures that defend the body from skin infections and monitor for tissue damage [1].

Immune responses can be divided into innate immunity and adaptive immunity.

Innate immunity

Innate immunity describes generalised measures to ward off infection. These include:

  • Physical barriers, such as the skin and mucous membranes, which stop the majority of microorganisms entering [2]
  • White blood cells, such as macrophages, which recognise microorganisms and release chemicals to stimulate and attract other immune cells
  • Neutrophils, which envelope and digest invading microorganisms by a process called phagocytosis (cellular ‘eating’).

Innate immune responses are often accompanied by inflammation [3].

Adaptive immunity

Adaptive immunity describes slower immune responses, including the production of immune cells that produce specific antibodies to target and remove a particular microorganism [1].

  • In cell-mediated immunity, T lymphocytes are produced that are conditioned to eliminate intracellular pathogens (viruses and bacteria) [4].
  • In humoral immunity, antibody-producing B lymphocytes deal with extracellular pathogens (bacteria in a polysaccharide capsule) [4].

Adaptive immunity results in the production of memory T lymphocytes (cells that have previously encountered an antigen and have “experience” fighting infections) and B lymphocytes (which produce antibodies) that are able to specifically target a particular infection. These lymphocytes continue to circulate and quickly recognise and remove the particular virus or bacteria when they are next encountered [3].

What is autoimmunity?

Autoimmunity is an immune response against the self that usually involves T and B lymphocytes. The particular protein or structure targeted by the T and B lymphocytes is called the self-antigen [3].

Autoimmunity may result in autoimmune disease with tissue damage or impaired physiological function. Autoimmune responses may also occur without causing disease [3].

Antibodies that react against self-antigens are called autoantibodies. In some autoimmune diseases, autoantibodies are the direct cause of tissue damage. In others, autoantibodies may be present without causing injury [5].

Examples of autoimmune diseases include:

Autoimmune skin diseases

Autoimmune blistering skin conditions diseases include:

Autoimmune bullous diseases

Autoimmunity and deregulation of the immune system also contribute to many skin diseases, such as:

Autoimmune skin diseases

Who gets autoimmune diseases?

Autoimmune diseases affect around 5% of the population [3].

  • Most autoimmune diseases are more common in women [3].
  • People with a family history of autoimmune disease are at higher risk of developing an autoimmune disease themselves [6].
  • Some studies show autoimmune diseases to be more common among patients from higher socioeconomic groups and northern latitudes [3].
  • Autoimmune blistering skin diseases are rare and have fairly similar rates in both men and women [7].

What causes autoimmune diseases?

Autoimmune disease occurs when the responses that normally prevent autoimmunity fail [5]. There are several protective mechanisms.

  • Maturing T lymphocytes in the thymus (a lymphoid organ found between the lungs where the T cells develop and mature) are removed if they react strongly against self-antigens [3].
  • Circulating T-regulatory cells suppress immune responses [5].
  • B lymphocytes that react strongly against self-antigens can undergo receptor editing and change their B-cell receptors [5].
  • T and B lymphocytes in the circulation need co-stimulation by other immune cells to become active [5].

The exact cause of a particular autoimmune disease is often not fully understood. Risk factors for many autoimmune diseases include genetic factors, infections, hormones, and drugs.

  • Genetic factors are most commonly polygenic (ie, multiple genes combine to increase risk) [1].
  • Infections may trigger an autoimmune process by mimicking a self-antigen or by increasing co-stimulatory molecules [1].
  • Genes on the Y chromosome may protect men from autoimmune disease, and oestrogen may play a role in the increased susceptibility of women to autoimmune diseases [1].
  • Certain drugs (eg, penicillamine, captopril, and vancomycin) can precipitate pemphigus vulgaris and pemphigus foliaceus [8].

Well-designed trials have concluded that there is no evidence that vaccines cause autoimmune disease [5].

Autoimmune blistering diseases

The autoimmune cause of this class of blistering skin conditions is confirmed by positive direct immunofluorescence microscopy revealing the deposition of antibodies in the skin.

Pemphigus

Pemphigus is a group of rare blistering disorders caused by circulating autoantibodies that bind to adhesion molecules in the skin, which disrupts keratinocytes from sticking together, causing intraepidermal blisters [9]. The main types of pemphigus are pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus [9].

  • Pemphigus vulgaris is characterised by blisters and erosions inside the mouth as well as on the skin. There are circulating autoantibodies against desmoglein 3, which is a protein important in keratinocyte cell-to-cell adhesion [10].
  • Pemphigus foliaceus causes superficial blisters typically on the trunk, scalp, and face. There are autoantibodies against desmoglein–1, a molecule that adheres skin cells to each other [3].
  • Paraneoplastic pemphigus causes blistering and ulceration in the mouth and sometimes the skin; it arises in association with malignancy, most often a non-Hodgkin lymphoma. The multiple autoantibodies seen in paraneoplastic pemphigus include those targeting desmoplakin proteins [11].

Pemphigoid

The pemphigoid group of diseases includes bullous pemphigoid, mucous membrane pemphigoid, and pemphigoid gestationis. Pemphigoid blisters are subepidermal and are caused by autoantibodies that bind in the area of the dermal-epidermal

junction [9].

  • Bullous pemphigoid predominantly affects older people who present with large tense fluid-filled blisters and erosions, often preceded by urticated or eczematous plaques [12]. There are antibodies against bullous pemphigoid antigen (BP180), a hemidesmosome-associated protein involved in keratinocyte to basement membrane adhesion, and against bullous pemphigoid antigen 230 (BP230), a protein found in basal keratinocytes [9].
  • Mucous membrane pemphigoid is characterised by recurrent blistering and ulceration of mucous membranes, particularly in the mouth and eyes, and can also affect the skin [9]. The split occurs lower in the dermal-epidermal junction, leading to scarring. Several autoantibodies have been associated with different presentations of mucous membrane pemphigoid including BP180, BP230, laminin 332, integrin alpha 6 and beta 4, and type VII collagen.
  • Pemphigoid gestationis affects women during pregnancy or shortly after delivery. Pemphigoid gestationis often starts as an intensely itchy urticaria-like rash around the belly button and then may spread to involve the entire skin surface, but not mucous membranes. It later typically progresses to tense blisters resembling those of bullous pemphigoid [13]. BP180 antibodies may be detected. The primary site of autoimmunity is thought to be the placenta.

Other rare blistering diseases

Dermatitis herpetiformis is an itchy blistering skin disease that typically affects the elbows, knees, and buttocks. It is associated with coeliac disease and the symptoms heal with a gluten-free diet [14]. It is characterised by blisters with a subepidermal deposition of immunoglobulin A (IgA) and a neutrophilic infiltrate. In dermatitis herpetiformis, the antibodies in the autoimmune response target the coagulation enzyme epidermal transglutaminase [15].

Linear IgA bullous dermatosis is a very rare autoimmune blistering disorder that can be acquired or drug-induced (eg, by vancomycin). The blisters are sometimes arranged in rings (known as the ‘pearl necklace’ sign) [9]. There is a subepidermal deposition of IgA antibodies which target a portion of the BP180 antigen, type VII collagen, or other basement membrane proteins.

Epidermolysis bullosa acquisita is also very rare. In its classical form, blisters and erosions form at areas of minor trauma [9]. In epidermolysis bullosa acquisita, the autoimmune reaction is directed against type VII collagen in the basement membrane zone of the skin and mucosa.

Bullous systemic lupus erythematosus is a rare presentation of subepidermal blistering in a patient with systemic lupus erythematosus.

How are autoimmune diseases in dermatology diagnosed?

A biopsy is usually needed for a definitive diagnosis of autoimmune skin disease, although a characteristic appearance may be suggestive of a particular condition [9].

Blood tests may include:

  • Tests for circulating skin autoantibodies (indirect immunofluorescence) [14]
  • Tests for coeliac antibodies, such as IgA tissue transglutaminase antibodies
  • Nonspecific tests for inflammatory markers (eg, C-reactive protein)
  • An autoimmune screen (eg, anti-nuclear antibodies).

Swabs may be taken of a ruptured blister to look for signs of a bacterial infection or herpes virus infection.

What is the differential diagnosis for autoimmune blistering skin diseases?

Blistering skin rashes associated with systemic illness may present with fever and ‘flu-like’ symptoms [14]. These disorders can include:

Other generalised blistering disorders that present with autoimmune skin disease symptoms include:

The blistering of mucous membranes is also a common symptom in these conditions:

What is the treatment for autoimmune skin diseases?

The treatment of autoimmune skin diseases depends on the specific condition.

Pemphigus and pemphigoid are mainly treated with systemic corticosteroids and immunosuppressive treatments [16].

Dermatitis herpetiformis is treated with dapsone and a gluten-free diet [15].

Systemic Lupus Erythematosus in Adults: Condition, Treatments, and Pictures – Overview

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Images of Lupus Erythematosus, Systemic

Overview

Systemic lupus erythematosus (SLE), also called lupus, is a chronic inflammatory disease that can affect almost any part of the body, especially the skin, joints, kidneys, heart, lungs, bones, blood, or brain. Systemic lupus erythematosus is considered an autoimmune disorder, meaning that a person’s own immune system attacks his or her own healthy cells and tissues, causing inflammation and damage.

Because systemic lupus erythematosus can affect any organ system, no two people have identical forms of the disease. However, most people with systemic lupus erythematosus report periods of time in which their symptoms seem to be mild or absent (remission) and other periods of time when the inflammation is more severe (flare or relapse).

Who’s at risk?

Systemic lupus erythematosus can occur in people of all ages, all races, and both sexes. However, it is far more common in women, especially those between 15–45 years old. In America, it is also more commonly seen in people with darker skin than in light-skinned people.

Although it is not directly inherited, lupus and other autoimmune diseases may run in families. Inheriting certain genes may make some people more susceptible to developing lupus.

In addition, certain environmental factors may trigger lupus in those who have a family (genetic) tendency toward the disease, including:

  • Ultraviolet light, especially sunlight
  • Certain medications, especially hydralazine and procainamide
  • Infections
  • Antibiotics, especially penicillins or sulfa-containing medicines
  • Stress
  • Hormonal changes, especially related to pregnancy and menstrual cycles

Signs and Symptoms

More than 90% of people with systemic lupus erythematosus have skin symptoms. The most common locations for the skin lesions of systemic lupus erythematosus include:

  • Face, especially cheeks and nose
  • Sun-exposed skin on arms, backs of hands, upper chest, and upper back due to increased sensitivity to sunlight (photosensitivity)
  • Fingers and fingernails
  • Mouth or nose
  • Scalp

The classic skin finding in systemic lupus erythematosus is the butterfly rash (malar blush). Redness across the cheeks and bridge of the nose can occur after sun exposure and may appear as much as several weeks before other symptoms develop.

A rash can develop in sun-exposed skin (photo-distribution), especially on the backs of the hands and fingers. This rash, which appears as red, scaly patches, can also affect the arms and trunk.

The skin around fingernails (nail folds) can be red and inflamed, and tiny, dilated blood vessels (telangiectasia) may be seen. In addition, people may develop Raynaud phenomenon, in which the fingers (and sometimes toes) turn pale and numb after exposure to cold temperatures.

Small, painless ulcers can develop in the nose or, more commonly, in the mouth, especially on the roof of the mouth.

When lupus affects the scalp skin, you may notice hair loss. It may be patchy, or there may be thinning across the scalp, especially at the temples.

In addition to the skin lesions of lupus, people may have:

  • Joint pain or swelling, especially in hands, wrists, and knees
  • Blood problems, including anemia and clotting disorders
  • Kidney disorders
  • Lung problems, such as painful breathing
  • Seizures or other brain disorders
  • Swollen lymph glands
  • Fever
  • Fatigue

Self-Care Guidelines

If you know you have systemic lupus erythematosus, several measures can help prevent flares:

  • Avoid intense sun exposure.
  • Apply sunscreen with SPF of 30 or higher every day.
  • Maintain healthy habits such as resting well, eating a balanced diet, and exercising regularly.
  • Reduce stress.
  • Avoid smoking and limit alcohol use.

When to Seek Medical Care

See your doctor if you develop a rash with fever and fatigue.

Treatments Your Physician May Prescribe

Lupus can be difficult to diagnose for 3 reasons: systemic lupus erythematosus can affect so many different organ systems, its symptoms can come and go, and no 2 people have exactly the same form of the disease. In addition to a careful review of your medical history, your doctor may perform blood tests, urinalysis, chest X-ray, or an electrocardiogram (ECG) before confirming the diagnosis of lupus.

If you have a rash that is suspicious for lupus, you nay need a skin biopsy. The procedure involves:

  1. Numbing the skin with an injectable anesthetic.
  2. Sampling a small piece of skin by using a flexible razor blade, a scalpel, or a tiny cookie cutter (called a “punch biopsy”). If a punch biopsy is taken, a suture or two may be placed and will need to be removed 6–14 days later.
  3. Having the skin sample examined under the microscope by a specially trained physician (dermatopathologist).

Even with a confirmed diagnosis of lupus, treatments vary as much as the disease itself. Treatments depend greatly on which organs are affected and how severe your symptoms are. In general, however, the following oral medications are frequently used for lupus:

  • Anti-malarial drugs such as hydroxychloroquine, chloroquine, or quinacrine
  • Corticosteroids
  • Anti-inflammatory medications such as aspirin, ibuprofen, naproxen, or indomethacin
  • Immune-suppressing medications including azathioprine, cyclophosphamide, methotrexate, cyclosporine, chlorambucil, or mycophenolate mofetil

As yet, there is no cure for lupus.

Trusted Links

MedlinePlus: LupusClinical Information and Differential Diagnosis of Lupus Erythematosus, Systemic

References

Bolognia, Jean L., ed. Dermatology, pp.594-595. New York: Mosby, 2003.

Freedberg, Irwin M., ed. Fitzpatrick’s Dermatology in General Medicine. 6th ed, pp.1619, 1677, 1681-1687. New York: McGraw-Hill, 2003.

Lupus – NHS

Lupus is a long-term condition that causes joint pain, skin rashes and tiredness. There’s no cure, but symptoms can improve if treatment starts early.

Non-urgent advice: See a GP if you often get:

  • joint and muscle pain
  • extreme tiredness that will not go away no matter how much you rest
  • rashes – often over the nose and cheeks

These are the main symptoms of lupus.

You might also have:

  • headaches
  • mouth sores
  • high temperature
  • hair loss
  • sensitivity to light (causing rashes on uncovered skin)

Information:

Coronavirus (COVID-19) update: how to contact a GP

It’s still important to get help from a GP if you need it. To contact your GP surgery:

  • visit their website
  • use the NHS App
  • call them

Find out about using the NHS during COVID-19

Important

Lupus is better managed if it’s found and treated early.

How lupus is diagnosed

Lupus, also called systemic lupus erythematosus, is not always easy to diagnose because it can be similar to other conditions.

Symptoms include inflammation of different parts of the body including the lungs, heart, liver, joints and kidneys.

The GP will usually do some blood tests. High levels of a type of antibody, combined with typical symptoms, means lupus is likely.

You might be referred for X-rays and scans of your heart, kidney and other organs if the doctor thinks they might be affected.

Once lupus is diagnosed, you’ll be advised to have regular checks and tests, such as regular blood tests to check for anaemia and urine tests to check for kidney problems.

Lupus can range from mild to severe

How lupus affects the body
Severity How it affects the body
Mild Joint and skin problems, tiredness
Moderate Inflammation of other parts of the skin and body, including your lungs, heart and kidneys
Severe Inflammation causing severe damage to the heart, lungs, brain or kidneys can be life threatening

Symptoms can flare up and settle down

Lupus often flares up (relapses) and symptoms become worse for a few weeks, sometimes longer.

Symptoms then settle down (remission). The reason why symptoms flare up or settle down is not known.

Some people do not notice any difference and their symptoms are constant.

Treatment for lupus

Lupus is generally treated using:

  • anti-inflammatory medicines like ibuprofen
  • hydroxychloroquine for fatigue and skin and joint problems
  • steroid tablets, injections and creams for kidney inflammation and rashes

Two medicines, rituximab and belimumab, are sometimes used to treat severe lupus. These work on the immune system to reduce the number of antibodies in the blood.

Versus Arthritis has more information on the treatments for lupus

Information:

Coronavirus advice

Get advice about coronavirus and lupus from Lupus UK

Living with lupus: things you can do yourself

Although medicines are important in controlling lupus, you can help manage your symptoms and reduce the risk of it getting worse.

Do

  • use high-factor (50+) sunscreen – you can get it on prescription if you have lupus

  • learn to pace yourself to avoid getting too tired

  • try to stay active even on a bad day

  • try relaxation techniques to manage stress – stress can make symptoms worse

  • wear a hat in the sun

  • tell your employer about your condition – you might be able to adjust your working pattern

  • ask for help from family, friends and health professionals

  • eat a healthy, balanced diet, including vitamin D and calcium

Don’t

  • do not smoke – stopping smoking is the most important thing to do if you have lupus

  • do not sit in direct sunlight or spend a lot of time in rooms with fluorescent lights

Lupus UK has support, advice and information for people with the condition.

Causes of lupus

Lupus is an autoimmune disease. This means the body’s natural defence system (immune system) attacks healthy parts of your body.

It’s not contagious.

The causes of lupus are not fully understood. Possible causes include:

  • viral infection
  • certain medicines
  • sunlight
  • puberty
  • childbirth
  • menopause

More women than men get lupus, and it’s more common in black and Asian women.

Pregnancy and lupus

Lupus can cause complications in pregnancy.

See a doctor before trying to get pregnant to discuss the risks and so your medicine can be changed if necessary.

Video: lupus in children

Watch this video to find out how lupus (an autoimmune condition) affects children.

Media last reviewed: 1 July 2021
Media review due: 1 July 2024

Page last reviewed: 23 September 2020
Next review due: 23 September 2023

Lupus-Specific Skin Disease and Skin Problems : Johns Hopkins Lupus Center

Most people with lupus experience some sort of skin involvement during the course of their disease. In fact, skin conditions comprise 4 of the 11 criteria used by the American College of Rheumatology for classifying lupus. There are three major types of skin disease specific to lupus and various other non-specific skin manifestautions associated with the disease.

Lupus-Specific Skin Disease

Three forms of specific skin disease occur in people with lupus, and it is possible to have lesions of multiple types. In addition, a person can also have one of the three forms outlined below without actually having full-blown systemic lupus erythematosus (SLE), but the presence of one of these disease forms may increase a person’s risk of developing SLE later in life. Usually, a skin biopsy is used to diagnose forms of cutaneous lupus, and various medications are available for treatment, including steroid ointments, corticosteroids (e.g., prednisone), and antimalarials (e.g., Plaquenil).

Chronic Cutaneous Lupus Erythematosus (CCLE) / Discoid Lupus Erythematosus (DLE)

Chronic cutaneous (discoid) lupus erythematosus is usually diagnosed when someone exhibits signs of lupus in the skin. People with SLE can also have discoid lesions, and about 5% of all people with DLE will develop SLE later in life. A skin biopsy is used to diagnose this condition, and the lesions have a characteristic pattern known to clinicians: they are thick and scaly, plug the hair follicles, appear usually on surfaces of the skin exposed to sun (but can occur in non-exposed areas), tend to scar, and usually do not itch.

If you are diagnosed with discoid lupus, you should try to avoid sun exposure when possible and wear sunscreen with Helioplex and an SPF of 70 or higher. In addition, you doctor may prescribe medications to help prevent and curb inflammation, including steroid ointments, pills, or injections , antimalarial medications such as Plaquenil, and/or immunosuppressive medications.

Subacute Cutaneous Lupus Erythematosus (SCLE)

About 10% of lupus patients have SCLE. The lesions characteristic of this condition usually do not scar, do not appear thick and scaly, and usually do not itch. About half of all people with SCLE will also fulfill the criteria for systemic lupus. Treatment can be tricky because SCLE lesions often resist treatments with steroid creams and antimalarials. People with SCLE should be sure to put on sunscreen and protective clothing when going outdoors in order to avoid sun exposure, which may trigger the development of more lesions.

Acute Cutaneous Lupus Erythematosus (ACLE)

Most people with ACLE have active SLE with skin inflammation, and ACLE lesions are found in about half of all people with SLE at some point during the course of the disease. The lesions characteristic of ACLE usually occur in areas exposed to the sun and can be triggered by sun exposure. Therefore, it is very important that people with ACLE wear sunscreen and protective clothing when going outdoors.

Common Lupus  Skin Problems

Malar Rash

About half of all lupus patients experience a characteristic rash called the malar or “butterfly” rash that may occur spontaneously or after exposure to the sun. This rash is so-named because it resembles a butterfly, spanning the width of the face and covering both cheeks and the bridge of the nose. The malar rash appears red, elevated, and sometimes scaly and can be distinguished from other rashes because it spares the nasal folds (the spaces just under each side of your nose). The butterfly rash may appear on its own, but some people observe that the appearance of the malar rash indicates an oncoming disease flare. Whatever the case, it is important to pay attention to your body’s signals and notify your physician of anything unusual.

Photosensitivity

50% of all people with lupus experience sensitivity to sunlight and other sources of UV radiation, including artificial lighting. For many people, sun exposure causes exaggerated sunburn-like reactions and skin rashes, yet sunlight can precipitate lupus flares involving other parts of the body. For this reason, sun protection is very important for people with lupus. Since both UV-A and UV-B rays are known to cause activation of lupus, patients should wear sunscreen containing Helioplex and an SPF of 70 or higher. Sunscreen should be applied everywhere, including areas of your skin covered by clothing, since most clothing items contain an SPF of only about 5. Be sure to reapply as directed on the bottle, since sweat and prolonged exposure can cause coverage to dissipate.

Livedo reticularis

People with lupus may experience a lacy pattern under the skin called livedo reticularis. This pattern may range anywhere from a violet web just under the surface of the skin to something that looks like a reddish stain. Livedo can also be seen in babies and young women, is more prominent on the extremities, and is often accentuated by cold exposure. The presence of livedo is usually not a cause for alarm, but it can be associated with antiphospholipid antibodies.

Alopecia

About 70% of people with lupus will experience hair loss (alopecia) at some point during the course of the disease. Hair loss in lupus is usually characterized by dry, brittle hair that breaks, and hair loss is more common around the top of the forehead. Physical and mental stress can also cause hair loss, as can certain medications, including corticosteroids such as prednisone. In many cases the hair will grow back, but hair loss due to scarring from discoid skin lesions may be permanent. There is no cure-all for hair loss, but treatments such as topical steroids and Rogaine may be prescribed. Sometimes dealing with the cosmetic side effects of lupus can be difficult, but some people find using hairpieces and wigs to be an effective means of disguising hair loss.

Oral and Nasal Ulcers

About 25% of people with lupus experience lesions that affect the mouth, nose, and sometimes even the eyes. These lesions may feel like small ulcers or “canker sores.” Such sores are not dangerous but can be uncomfortable if not treated. If you experience these types of lesions, your doctor may give you special mouthwash or Kenalog in Orabase (triamcinolone dental paste) to help expedite the healing process.

Raynaud’s Phenomenon

Approximately one-third of all people with lupus experience a condition called Raynaud’s phenomenon in which the blood vessels supplying the fingers and toes constrict. The digits of people with Raynaud’s are especially susceptible to cold temperatures. Often people with the condition will experience a blanching (loss of color) in the digits, followed by blue, then red discoloration in temperatures that would only be mildly uncomfortable to other people (such as a highly air-conditioned room). It is very important that people with Raynaud’s wear gloves and socks when in air-conditioned spaces or outside in cool weather. Hand warmers used for winter sports (e.g., Hot Hands) can also be purchased and kept in your pockets to keep your hands warm. These measures are very important, since Raynaud’s phenomenon can cause ulceration and even tissue death of the fingers and toes if precautions are not taken. People have even lost the ends of their fingers and toes due to the poor circulation involved in Raynaud’s phenomenon. Cigarettes and caffeine can exacerbate the effects of Raynaud’s, so be sure to avoid these substances. If needed, your doctor may also recommend a calcium channel blocker medication such as nifedipine or amlodipine to help dilate your blood vessels.

Hives (Urticaria)

About 10% of all people with lupus will experience hives (urticaria). These lesions usually itch, and even though people often experience hives due to allergic reactions, hives lasting more than 24 hours are likely due to lupus. If you experience this condition, be sure to speak with your doctor, since s/he will want to be sure that the lesions are not caused by some other underlying condition, such as vasculitis or a reaction to medication. Your doctor will probably distinguish these lesions from those caused by vasculitis by touching them to see if they blanch (turn white).

Purpura

Approximately 15% of people with lupus will experience purpura (small red or purple discolorations caused by leaking of blood vessels just underneath the skin) during the course of the disease. Small purpura spots are called petechiae, and larger spots are called eccymoses. Purpura may indicate insufficient blood platelet levels, effects of medications, and other conditions.

Cutaneous Vasculitis

Some people with lupus may develop a condition known as cutaneous vasculitis, in which the blood vessels near the skin experience inflammation that ultimately restricts blood flow. This condition can cause hive-like lesions on the skin that may itch and do not turn white when depressed. Other skin abnormalities may also be present, including actual gangrene of the digits. If left untreated, vasculitic lesions may cause ulceration and necrosis (cell death), and dead tissue must be surgically removed. Rarely, fingers or toes with aggressive ulceration and gangrene may require amputation. Therefore, it is very important that you notify your doctor of any skin abnormalities.

Dermatomyositis | Cedars-Sinai

Not what you’re looking for?

What is dermatomyositis?

Dermatomyositis is a rare disease that causes muscle inflammation and skin rash. It’s
one of a group of muscle diseases that cause muscle inflammation, weakness, and
swelling. It’s different from other muscle diseases because it also causes inflammatory
skin rashes. Dermatomyositis is the term used to describe both muscle and skin symptoms,
but some people will have only one or the other.

It
can occur at any age, but it most often affects adults ages 50 to 70. Women are twice as
likely as men to be diagnosed with the disease. Some people with the disease also have a
connective tissue disorder, such as lupus or rheumatoid arthritis.

What causes
dermatomyositis?

The
exact cause is not known. But dermatomyositis can be linked to cancer, other autoimmune
diseases, infections, or genetic problems.

What are the symptoms of
dermatomyositis?

Swelling and inflammation in the blood vessels that supply your skin and muscles cause symptoms such as:

  • Red or purple rash on sun-exposed areas that may be painful or itchy
  • Red or purple swelling of the upper eyelids (heliotrope)
  • Red or purple spots on the knuckles, elbows, knees, and toes (Gottron papules)
  • Joints that feel stiff and turn pale and painful in cold conditions and feel better when warmed (Raynaud phenomenon)
  • Scaly, rough, dry skin, which can lead to thinning hair
  • Swollen, red areas around the fingernails
  • Hard lumps under the skin caused by calcium deposits (calcinosis)
  • Muscle weakness in the neck, hip, back, and shoulders
  • Trouble swallowing and voice changes
  • Tiredness, fever, and weight loss
  • Muscle aches
  • Trouble rising from a chair or getting out of bed due to muscle weakness

Sometimes the muscle inflammation can spread to other parts of the body including the heart, gastrointestinal tract, and lungs. Lung involvement can cause breathing trouble and coughing. Adults may have a low-grade fever, along with lung inflammation and sensitivity to light.

How is dermatomyositis
diagnosed?

First your healthcare provider will review your health history and do a physical exam.
 He or she will look for an underlying disease, such as cancer. Your provider may also
do the following tests:

  • Blood
    tests.
    These are done to look for signs of muscle inflammation. They also
    check for abnormal proteins that form in autoimmune disease. The most common blood
    tests include muscle enzyme creatine kinase and the antinuclear antibody.
  • Electromyelogram (EMG). This may be done to find abnormal electrical
    activity in affected muscles.
  • MRI.
    This test uses a magnetic field and radio waves to create detailed images of
    tissues.
  • Skin or
    muscle biopsy.
    Tiny pieces of muscle tissue are taken and looked at under a
    microscope.

How is dermatomyositis
treated?

Treatment will depend on your symptoms, your age, and your general health. There’s no
cure for the condition, but the symptoms can be managed. You may need more than one kind
of treatment. And your treatment may need to be changed over time. Treatments
include:

  • Physical
    therapy.
    Special exercises help to stretch and strengthen the muscles.
    Orthotics or assistive devices may be used.
  • Skin
    treatment.
    You may need to avoid sun exposure and wear sunscreen to help
    prevent skin rashes. Your healthcare provider can treat itchy skin rashes with
    antihistamine drugs or with anti-inflammatory steroid creams for the skin.
  • Anti-inflammatory medicines. These are steroid drugs, or corticosteroids.
    They ease inflammation in the body. They may be given by mouth or through an IV.
  • Immunosuppressive medicines. These medicines block or slow down your body’s
    immune system. 
  • Immunoglobulin. If you have not responded to other treatments, your provider
    may prescribe these medicines. They are donated blood products that may boost your
    body’s immune system. They are put directly into your bloodstream through an IV.
  • Surgery.
    You may need surgery to remove the calcium deposits (calcinosis) under the skin
    if they become painful or infected.

Talk
with your healthcare providers about the risks, benefits, and possible side effects of
all medicines.

What are possible complications of
dermatomyositis?

Possible complications for some people with dermatomyositis include lung disease, heart
disease, or cancer. These can make treatment more difficult.

Living with
dermatomyositis

If
you have dermatomyositis, you may need treatment for the rest of your life. It’s
important to learn as much as you can about the disease. Work closely with your
healthcare provider. Researchers are studying causes and treatments for the condition.
Early diagnosis and treatment for the disease may improve over time.

When should I call my healthcare
provider?

If
your symptoms get worse or you notice new symptoms, notify your healthcare provider.

Key points about
dermatomyositis

  • Dermatomyositis is a rare disease that causes muscle weakness and skin rash.
  • Symptoms include a red or purple rash on sun exposed skin and eyelids, calcium
    deposits under the skin, muscle weakness, and trouble talking or swallowing.
  • There is
    no cure, but treatment is done to reduce the symptoms.
  • Complications include lung disease, heart disease, and cancer.

Next steps

Tips
to help you get the most from a visit to your healthcare provider:

  • Know
    the reason for your visit and what you want to happen.
  • Before
    your visit, write down questions you want answered.
  • Bring
    someone with you to help you ask questions and remember what your provider tells
    you.
  • At the
    visit, write down the name of a new diagnosis, and any new medicines, treatments, or
    tests. Also write down any new instructions your provider gives you.
  • Know
    why a new medicine or treatment is prescribed, and how it will help you. Also know
    what the side effects are.
  • Ask if
    your condition can be treated in other ways.
  • Know
    why a test or procedure is recommended and what the results could mean.
  • Know
    what to expect if you do not take the medicine or have the test or procedure.
  • If you
    have a follow-up appointment, write down the date, time, and purpose for that
    visit.
  • Know how
    you can contact your provider if you have questions.

Medical Reviewer: Diane Horowitz MD

Medical Reviewer: Rita Sather RN

Medical Reviewer: Raymond Kent Turley BSN MSN RN

© 2000-2021 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional’s instructions.

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Ask the Expert: Skin Rashes and Sjögren’s

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Skin rashes most certainly occur in the setting of Sjögren’s. As opposed to many of the other autoimmune skin rashes that I see in my autoimmune dermatology clinic, there are no rashes that are specific for Sjögren’s. In other words, I cannot look at a rash in a Sjögren’s patient and immediately tell them that they have Sjögren’s. In comparison, I can often look at a lupus rash and tell the patient they have underlying lupus. That being said, Sjögren’s patients exhibit a variety of skin rashes that are important to recognize and know when to see a board-certified dermatologist.

By and far the most common skin conditions associated with Sjögren’s are xerosis, or clinically dry skin, and eczematous dermatitis. These are caused primarily by barrier dysfunction of the skin. Gentle skin care practices and liberal moisturizers are the best way to prevent these common skin eruptions. When caring for your skin you should take no more than one short, lukewarm shower per day. You should remain in the shower for less than 10 minutes. Soaps like Dove sensitive and VanicreamTM, can be utilized as they will not strip your skin. If a wash cloth is used while bathing, it should be limited to areas that give off body odor like the armpits, genital, and buttock areas, in order to not disrupt the skin barrier. Immediately after getting out of the shower, you should pat dry and then apply a moisturizer. Ointments are the best moisturizers followed by creams. Lotions are not typically moisturizing enough for patients with Sjögren’s. The best ointments are Vaseline® or Aquaphor®. If an ointment is too greasy for your taste, cream moisturizers like CeraVe®, VanicreamTM, Eucerin®, or Aveeno®can be used. For itchy skin, Sarna Original lotion can give immediate relief especially when stored in the refrigerator. If scaly, dry skin is present, utilizing moisturizers with lactic acid, urea, or salicylic acid can be used. Examples include: AmLactin®, CeraVe® SA, and Eucerin® Roughness Relief. If your rash is not responding to moisturizers and gentle skin care practices, a prescription corticosteroid cream or a steroid-sparing anti-inflammatory cream may need to be prescribed by your physician.

If you develop a purple-to-red rash that does not lighten, or blanch, with pressure, ulcerations of the skin, or a purple net pattern on your skin, this should prompt you to see a dermatologist for evaluation. Rashes that are associated with purpura (blood spots) can represent vasculitis, an inflammation of the blood vessels. Although these rashes are not as common as the ones discussed above, they are important to recognize in Sjögren’s patients and should be seen quickly by a dermatologist.

Natalie Wright, MD, FAAD

This information was first printed in the Foundation’s patient  newsletter for members. 

90,000 What is the danger of lupus, which was found in Julia Nachalova

One of the diseases that, according to media reports, led to the death of singer Yulia Nachalova, was systemic lupus erythematosus. Gazeta.Ru has figured out what kind of disease it is and how dangerous it is.

On March 16, singer Yulia Nachalova died. According to media reports, one of the diseases that caused her death was systemic lupus erythematosus.

Systemic lupus erythematosus is a chronic autoimmune disease of the connective tissue and blood vessels.With it, antibodies produced by the immune system damage healthy cells. The disease got its name because of its characteristic feature – a rash on the bridge of the nose and cheeks, which, as it was believed in the Middle Ages, resembles the places of wolf bites.

However, a rash appears only in 30-50% of patients, and dermatological manifestations in general – in about 65% of cases. Thick red scaly spots may appear on the arms and trunk, which can subsequently lead to skin atrophy, and among the possible manifestations of the disease are ulcers on the mucous membranes of the mouth and vagina, trophic ulcers.

Many patients complain of joint pain, mainly in the wrists and small joints of the hands.

The disease can lead to deformation of the joints, in 20% of cases the changes are irreversible.

The disease affects the lungs, causing pneumonia and pleurisy, the cardiovascular system suffers from pericarditis, myocarditis, Liebman-Sachs endocarditis (inflammatory diseases of various parts of the heart). Lupus patients also develop atherosclerosis more often and faster. When the gastrointestinal tract is affected, stomatitis, enlargement of the esophagus, liver damage, ischemia of the stomach wall are detected.Nephritis, an inflammatory disease of the kidneys, is also common with lupus.

Possible damage to the nervous system – movement disorders, headaches, meningitis, convulsive disorders. One of the most dangerous neurological manifestations of lupus is demyelinating syndrome, in which the lining of nerve cells is destroyed. This leads to a deterioration in the conduction of signals in the affected nerves. Depending on the location, the syndrome can cause a wide range of complications, from tremors and impaired coordination of movements to blindness and paralysis.

Of the nonspecific symptoms, patients are concerned about unreasonable rises in temperature, weakness, headaches, muscle pains, and fatigue.

Lupus affects life expectancy.

Survival rate in 10 years after diagnosis is 80%, in 20 years – 60%.

The main causes of death are nephritis and neurological manifestations of the disease, as well as infections that developed against the background of the underlying disease. There are cases of survival of 25-30 years.

Lupus cannot be cured, but symptoms may be alleviated.For this, glucocorticosteroids, stem cell therapy, and immunosuppressants are used. In case of complications, symptomatic treatment is used.

Note that Yulia Nachalova’s doctor denies that she has lupus erythematosus.

Nevertheless, the disease also affects abroad – in particular, the singer Lady Gaga, Toni Braxton, Selena Gomez. The latter, due to illness, survived a kidney transplant, the organ was donated to her by a friend, actress Francia Rice.

Mothers with lupus give birth to children with 90,033 autism , twice as often as other women,

was found by American scientists from Mount Sinai Hospital in New York.

They came to similar conclusions based on the results of a study of 509 women with lupus and their 719 children, as well as 5900 women without lupus and their 8500 children.

At the same time, scientists associate the development of autism in a child with the time of conception. In their opinion, it should not occur during the active manifestation of the disease.

Previously identified gene , which affects the complications of lupus. It turned out that the inactive protein ABIN1 not only makes the human body defenseless against disease, but also contributes to the deactivation of pathways that prevent the inflammatory process.

In addition, scientists were able to link the functioning of this protein with the subsequent influence on the transcription factor NF-βB, which in the future will effectively fight the disease.

Russian scientists have developed a prototype of a fundamentally new drug for the treatment of autoimmune diseases, including lupus.

They were able to turn off the TNF protein’s ability to increase the risk of severe disease, while retaining its ability to fight tumors and protect against tuberculosis.In experiments on mice, the researchers were able to prove the effectiveness of this approach.

“Based on these results, the biomedical industry can design and test fundamentally new drugs for the treatment of autoimmune diseases,” said Sergei Nedospasov, one of the researchers.

90,000 Lupus Symptoms and Oral Health

Lupus is an autoimmune disease or disease that occurs due to a malfunction of the immune system.Lupus can affect oral health by developing symptoms such as acute (sudden onset and short duration) or chronic (persistent over a long period of time) soft tissue lesions. The disease is not transmitted from person to person even with bodily contact; the reason for its development is that the body perceives some of its own tissues as foreign. The immune system protects organs from the effects of various cells, in particular, antibodies protect the body from infectious agents.Symptoms of the disease occur when antibodies begin to infect normal cells in the body. The medical name for this disease is systemic lupus erythematosus, or other forms of lupus erythematosus. Symptoms of the disease can be combined with those of other autoimmune diseases and may include a skin rash on the nose and cheeks called a butterfly rash. The rash can appear on other areas of the skin that are often exposed to sunlight.Other symptoms include fatigue, joint swelling, fever, hair loss, and inflammation of organs, including the heart and kidneys.

Who is affected by the disease?

According to the Lupus Research Foundation of America, the disease affects women more often than men. In women, the disease usually develops during childbearing age, that is, between 20 and 45 years, while women of African American, Spanish and Asian descent are at greatest risk.The Foundation also reports that there are 16,000 new cases of lupus every year and that 5 million people worldwide are affected by the disease.

Diagnosis

There is no separate diagnostic method for this disease. The diagnosis is made by ruling out other types of immune diseases and based on a number of symptoms characteristic of the disease, such as a butterfly rash on the face, other manifestations of skin rashes, inflammation of the heart tissue, kidney problems, arthritis, immune system disorders, and mouth ulcers.

Diagnosed changes in the state of the oral cavity

This disease does not contribute to the development of two main diseases of the oral cavity: dental caries and periodontal disease. However, according to an article published in the European Journal of Dermatology, 9–45 percent of patients with this condition may develop mouth sores or other lesions of the oral mucosa. Such ulcers and lesions can be acute or chronic and look similar to other types of intraoral lesions.Biopsy and examination by a rheumatologist may be required to initially diagnose the disease and to confirm that intraoral lesions are caused by lupus. In the event that the disease affects the salivary glands, the patient may experience dry mouth, which will require fluoride treatment. Treatment of intraoral lesions is mitigating and often involves the use of topical steroids.

Available treatments

The choice of treatment method depends on the affected organ and the severity of the disease.Treatment may include the use of steroids, antimalarials, and pain relievers. Lifestyle changes and stress management will also be required. For more information on treatments, visit the Mayo Clinic website. Support measures are also available for people with this condition. In addition, there are a number of experimental drugs, such as rituximab, that help rebuild the immune system, thus reducing the manifestation of the disease.However, lupus, like all autoimmune diseases, is incurable; only long-term treatments are available. If you have or suspect lupus, please inform your dental hygienist and dentist at your next check-up.

90,000 Treatment of lichen skin in adults: symptoms, types, cost of treatment

Trichologist, dermatologist, doctor of the highest category

Moskovsky prospect, 143

Dermatologist oncologist, doctor of the highest category

Grazhdansky prospect, d.107, building 4

Dermatologist oncologist, doctor of the highest category

Grazhdansky prospect, 107, office 4

Kolomyazhsky prospect, 20

Dermatologist, specialist in laser technologies in oncodermatology, doctor of the highest category

Kolomyazhsky prospect, 20

Dermatologist, specialist in laser technologies in oncodermatology, doctor of the highest category

Moskovsky prospect, 143

Dermatologist, specialist in laser technologies in oncodermatology

Moskovsky prospect, d.143

Grazhdansky prospect, 107, office 4

Kolomyazhsky prospect, 20

Dermatovenereologist, trichologist, cosmetologist, laser technology specialist

Moskovsky prospect, 143

Grazhdansky prospect, 107, office 4

Kolomyazhsky prospect, 20

Dermatovenereologist, trichologist, cosmetologist. Highest qualification category.

Moskovsky prospect, 143

Dermatologist, cosmetologist, Laser technology specialist in oncodermatology

Kolomyazhsky prospect, d.20

Dermatovenereologist, specialist in laser technologies in oncodermatology

Moskovsky prospect, 143

Kolomyazhsky prospect, 20

dermatologist, cosmetologist, laser technology specialist

Grazhdansky prospect, 107, building 4

Dermatovenereologist, Doctor of Medicine, Professor

Moskovsky prospect, 143

Dermatovenereologist, specialist in laser technologies in oncodermatology

Grazhdansky prospect, d.107, building 4

Oncologist-dermatologist, candidate of medical sciences

Moskovsky prospect, 143

Grazhdansky prospect, 107, office 4

Kolomyazhsky prospect, 20

Puzyrchatka – KVD No. 2

True pemphigus is a chronic disease of an autoimmune nature, which is characterized by the appearance of blisters on clinically healthy skin and mucous membranes. The clinical course of pemphigus has allowed clinicians to identify the following forms of the disease: vulgar, erythematous, vegetative and leaf-shaped.Pemphigus is diagnosed by detecting acantholytic cells in a smear-imprint and histological detection of bubbles located inside the epidermis. In the treatment of pemphigus, the main course is the appointment of glucocorticosteroids, it is successfully combined with the methods of extracorporeal hemocorrection: plasmaphoresis, hemosorption, cryoapheresis.

True pemphigus is a chronic autoimmune disease characterized by the appearance of blisters on clinically healthy skin and mucous membranes.

Causes of pemphigus

The most probable cause of pemphigus is disorders of autoimmune processes, as a result of which the cells of the body become antibodies for the immune system. Violation of the antigenic structure of epidermal cells occurs under the influence of external factors, in particular the effect of retroviruses and aggressive environmental conditions.

The damaging effect on the cells of the epidermis and the production of specific antigens leads to disruption of communication between cells, resulting in the formation of bubbles.Risk factors for pemphigus have not been established, but in individuals with a hereditary predisposition, the incidence rate is higher.

Clinical manifestations of pemphigus

Pemphigus has a long undulating course, and the lack of adequate treatment leads to a violation of the general condition of the patient.

At vulgar form pemphigus blisters are localized throughout the body, have different sizes and are filled with serous contents, while the tire on the bladders is flaccid and thin.

Pemphigus vulgaris debuts, as a rule, on the mucous membrane of the mouth and nose, and therefore patients receive therapy from dentists and otolaryngologists for a long time and unsuccessfully. At this stage of pemphigus, patients complain of pain while eating and talking, hypersalivation and a specific bad breath. The duration of this period is from three months to a year, after which the pemphigus becomes widespread and the skin is involved in the inflammatory process.

Sometimes patients do not notice the presence of blisters due to their small size and thin lining, the blisters open quickly, and therefore the main complaints of patients with pemphigus at this period are painful erosions. Long-term and unsuccessful therapy of stomatitis is carried out before pemphigus is diagnosed. Blisters that are localized on the skin tend to spontaneously open with exposure of the eroded surface and with the remnants of the tire, which shrinks into crusts.

Pemphigus erosions are bright pink, with a smooth glossy surface, differ from erosions in other diseases by a tendency to peripheral growth and generalization with the formation of extensive lesions.If the pemphigus takes such a course, then the general condition of the patient worsens, intoxication develops, a secondary infection joins and without proper treatment such patients die.

With pemphigus vulgaris, Nikolsky’s syndrome is positive in the lesion focus and sometimes on healthy skin – with a slight mechanical effect, detachment of the upper layer of the epithelium occurs.

Pemphigus erythematosus differs from vulgar pemphigus in that at the beginning the skin is affected; erythematous lesions on the chest, neck, face and on the scalp are seborrheic in nature, have clear boundaries, the surface is covered with yellowish or brown crusts of varying thickness.If these crusts are separated, then an eroded surface is exposed.

With erythematous pemphigus, the blisters can be small, their lining is flabby and flaccid, they spontaneously open up very quickly, therefore it is extremely difficult to diagnose pemphigus. Nikolsky’s symptom, as with erythematous pemphigus, can be localized for several years, then, with the generalization of the process, it acquires vulgar features.

Pemphigus erythematosus should be differentiated from lupus erythematosus and seborrheic dermatitis.

Pemphigus is clinically manifested by erythema-squamous rashes, thin-walled blisters tend to appear on previously affected areas, after opening of the blisters, a bright red eroded surface is exposed, upon drying of which lamellar crusts are formed. Since with this form of pemphigus, bubbles also appear on the crusts, the affected skin is sometimes covered with a massive layered crust due to the constant separation of exudate. Pemphigus leaf affects the skin, but in very rare cases, mucosal lesions are observed, it quickly spreads throughout the healthy skin and at the same time there are blisters, crusts and erosion on the skin, which merge with each other to form an extensive wound surface.Nikolsky’s symptom is positive even on healthy skin; with the addition of pathogenic microflora, sepsis develops, from which the patient usually dies.

Vegetative pemphigus is more benign, sometimes patients are in a satisfactory condition for many years. Blisters are localized around natural openings and in the area of ​​skin folds. Opening, the bubbles reveal erosion, at the bottom of which soft vegetation with a fetid odor is formed; vegetation from above covered with serous or serous-purulent bloom.There are pustules on the periphery of the formations, and therefore the vegetative pemphigus must be differentiated from the vegetative chronic pyoderma. Nikolsky’s syndrome is positive only near the affected skin, but in the terminal stages, the vegetative pemphigus is similar to the vulgar pemphigus in its clinical manifestations.

Diagnostics of all types of pemphigus

Clinical manifestations, especially at the initial stages of the disease, are not very informative, and therefore the questioning of the patient helps to avoid an erroneous diagnosis.Laboratory tests allow one to suspect pemphigus, so acantholytic cells are found in smears-prints during cytological examination. Histological examination reveals the intraepidermal arrangement of the blisters.

Principles of pemphigus treatment

A hypoallergenic diet and the elimination of rough food, canned food, simple carbohydrates, salty foods and other extractive substances from the diet are indicated for patients with any form of pemphigus. If the oral cavity is affected, then it is necessary to include in the diet of soups-mashed potatoes and mucous cereals in order to prevent a complete rejection of food, food rich in protein, included in the diet, accelerate the process of cell regeneration and epithelization of open erosion.

All patients with pemphigus should be monitored by a dermatologist, a gentle mode of work, lack of physical activity and avoidance of insolation are recommended. Frequent change of underwear and bedding prevents secondary infection.

The appointment of glucocorticosteroids at once in high doses is shown, since otherwise the therapeutic effect will not be achieved, after the relief of acute manifestations of pemphigus, the dosage of hormonal drugs is gradually reduced to the minimum effective.In the treatment of pemphigus, methods of extracorporeal hemocorrection are used: hemosorption, cryoapheresis and membrane plasmapheresis.

Aniline dyes and non-aggressive antiseptic solutions are used as topical treatment for pemphigus.

The prognosis of pemphigus is always unfavorable, since in the absence of adequate treatment, the death of patients occurs rather quickly from complications that have joined. Long-term hormone therapy in high doses increases the risk of side effects, but pemphigus begins to recur when glucocorticosteroids are withdrawn.

Pemphigus of newborns

Pemphigus of newborns is an acute, highly contagious infection of the skin, clinically manifested in the form of pustules, which very quickly spread over the skin.

Unlike true pemphigus, pemphigus of newborns is bacterial in nature and its causative agent is Staphylococcus aureus.

In the pathogenesis of pemphigus of newborns, an important place is occupied by the reactivity of the skin of newly born children, which increases with birth trauma, prematurity and with the wrong lifestyle of the pregnant woman.In response to the action of bacterial factors, blisters form on the skin and pemphigus of newborns is diagnosed.

In the epidemiology of neonatal pemphigus, there is a violation of hygiene standards in maternity hospitals, the presence of foci of chronic infection in the staff, autoinfection with pemphigus is possible if the newborn develops purulent diseases of the umbilicus.

Pemphigus of newborns occurs in the first days of a child’s life, but the disease is possible 1-2 weeks later. On clinically healthy or slightly erythematous skin, small, tense, thin-walled blisters with serous contents appear.After a few hours, the process generalizes, the bubbles increase in size and open. In place of the blisters, painful erosions remain with remnants of the epidermis along the edges, the erosion is covered with serous-purulent crusts. With pemphigus of newborns, children have symptoms of intoxication, fever, many of them refuse to eat.

In the absence of adequate treatment, pemphigus of newborns provokes inflammatory processes of internal organs: pneumonia, otitis media, phlegmon.In weak and deeply premature babies, a septic form of pemphigus is possible, the mortality rate in which is quite high.

Diagnose pemphigus of newborns on the basis of visual examination, it must be differentiated from syphilitic pemphigus, which is a manifestation of congenital syphilis, in which the bubbles are localized on the palms.

Antibitotic therapy made it possible to significantly reduce the mortality rate from pemphigus in newborns, whereas earlier more than half of sick children died, the prognosis of pemphigus in newborns is favorable with timely and adequate therapy.Aniline dyes and non-aggressive antiseptics are used locally.

Preventive measures are the change of bed and underwear, removal from work of staff with pustular eruptions, supervision of pregnant women and timely local therapy for mothers of pustular rash.

90,000 Clinical variants of purple eruptions

Authors:
L. D. Kalyuzhnaya, MD, Professor of the Department of Dermatovenereology, National Medical Academy of Postgraduate Education named after P.L. Shupika, Kiev

In dermatology, which is characterized by a large number of diagnostic options, involvement in a number of syndromes, a significant proportion of changes in the skin are related to diagnostic signs of internal diseases. The immediate appointment of topical agents, without excluding possible systemic pathology, has receded into the distant past.Moreover, it is skin rashes that often serve as the first signal of general ill health.

Recently, cases of vasculitis of the skin, including in the form of purples, have become much more frequent. Purpura is a hemorrhagic rash on the skin and mucous membranes that can manifest as petechiae, macular purpura, ecchymosis, palpable purpura, non-inflammatory and inflammatory reticular purpura. The first three forms differ in the size of the elements. The cause of purple can be both small vessel blockage syndrome and vasculitis.Elucidation of the factors contributing to the onset of purpura makes it possible to distinguish different approaches to diagnosis and treatment.

Microvascular blockage syndrome is based on a non-inflammatory factor. At an early stage of the process, erythema is occasionally determined, and over time, purpura or necrosis appears, which covers about two-thirds of the affected area. In the case of macular petechial lesions <4 mm in diameter, the cause may be due to thrombocytopenia hemostasis (<50 thousand / mm ³ ) or impaired platelet function.Along with idiopathic thrombocytopenic purpura, drug-induced thrombocytopenia is also included (for example, with chemotherapy), as well as with bone marrow insufficiency in the form of infiltration, fibrosis. If purple is based on an altered platelet function, then, as a result, a congenital or hereditary functional defect of platelets, as well as an acquired defect provoked by aspirin, renal failure, and monoclonal gammopathy, can manifest itself. In addition, secondary thrombocytosis is possible in myeloproliferative diseases (often> 1 million / mm ³ ).

Meanwhile, similar rashes can occur not only in connection with thrombotic pathology. The reason for their appearance may be a sharp rise in intravascular pressure caused by an attack of paroxysmal cough, repeated vomiting, childbirth, stroke, “jumping” blood pressure, ligature. Petechial rashes can also appear with trauma, vitamin C deficiency, pigmented purpura, hypergammaglobulinemic purpura. In the differential diagnosis of intermediate purpura (5-9 mm in diameter), the following etiological factors can be established: hypergammaglobulinemia, the presence of infection or inflammation in thrombocytopenia, and occasionally vasculitis of small vessels.In the case of ecchymosis (> 1 cm in diameter), a procoagulant defect with minor trauma should be assumed. The reasons may also be the intake of anticoagulants, liver failure with reduced procoagulant synthesis, vitamin K deficiency, and occasionally disseminated intravascular coagulation. At the same time, one should take into account the possible reduced dermal support of the vessels, which can manifest itself in a number of chronic dermatoses, especially with a defect in the connective tissue.Such purple manifestations can be expressed with actinic purpura (both solar and senile), topical and systemic corticosteroid therapy, vitamin C deficiency, systemic amyloidosis, Ehlers-Danlos syndrome.

As a rule, the so-called obvious inflammatory purpura with early erythematous manifestations should be considered the most severe both in its course and in the prognosis. First of all, this should include leukocytoclastic vasculitis caused by diseases of the immune complexes.They can manifest as changes only in small vessels, including those provoked by infections or medications, IgA- or IgG- and IgM-complexes. Leukocytoclastic vasculitis of the skin is characterized initially by the manifestation of obvious purpura, then urticaria, hemorrhagic spots or vesicles. Favorite localization – the lower extremities, especially the ankles, postcapillary venules are predominantly affected. At first, the changes resemble erythema multiforme, they are often localized at the sites of injury or wearing tight clothing.Then there are signs of a transition to a systemic nature in the form of weight loss, arthralgia and myalgia, fever. In almost 90% of patients, the disease resolves after a few weeks or months, in 10% it takes on a chronic relapsing course. If medium-sized vessels are also involved, mixed cryoglobulinemia or rheumatic vasculitis (lupus erythematosus or rheumatoid arthritis) may be detected. Non-leukocytoclastic vasculitis should include possible purple eruptions with erythema multiforme, varioliform or lichenoid pityriasis, pigmented purpura, Waldenstrom’s hypergammaglobulinemic purpura.

The formation of speckled purpura is caused by simple hemorrhage with erythrocyte extravasation and minimal signs of inflammation. The evolution of these manifestations is expressed in the successive clarification of cells and hemoglobin; clinically manifests itself by changing the color of the rash: from red-blue or purple (through green, yellow, brown) to complete disappearance of the color.

In inflammatory hemorrhage syndromes such as small vessel vasculitis or microvascular blockage, evolution is more complex.Hemostasis is determined by the formation of a plug, which can correspond to the lumen of a small vessel or increase to the size of a thrombus. The size of the clot determines the perspective of this process; it can even provoke death by thrombosis, embolism and necrosis. Controlling the formation of a clot is extremely important because the formation of a small clot can be fatal from bleeding. A clot inappropriate to the diameter of the vessel leads to thrombosis, embolism and necrosis, lack of control over it leads to fibrinolysis, and then to thrombosis and bleeding, intravascular coagulation.Natural anticoagulant control is provided by certain complexes, primarily a tissue factor inhibitor and antithrombin. Another essential natural anticoagulant system is thrombomodulin – protein C – protein S. Antithrombin III and protein C are involved in the function of large vessels, and protein C – in the normal function of microvessels. Clot-derived thrombin can bind thrombomodulin on the endothelial cell surface. Thrombin associated with thrombomodulin loses its ability to activate procoagulant factors, binds and activates protein C and anticoagulant vitamin K-dependent protein.

Special attention should be paid to idiopathic purpura (progressive pigmentary purpura, selected purpura syndromes). Idiopathic purpura is known as progressive pigmented purpura. The etiology of idiopathic purpura is unclear. Some favorable localized purpura occur as a result of increased intravascular pressure, impaired vascular permeability. In addition, these purpura are considered as a preliminary process of the formation of leukocyte inflammatory vasculitis.Separate purple syndromes are distinguished, first of all, they are pigmented purple rashes. When they are designated, synonyms such as capillaritis, chronic pigment purpura, simple purpura, Shamberg-Mayocchi disease are often used. In addition, in the literature variants of synonyms are possible, denoting the same process: Shamberg’s disease, Shamberg’s purpura, Shamberg’s progressive pigment dermatosis, progressive pigmentary purpura; purpura annular telangiectatic Mayocchi, Mayocchi’s disease; Guzhero-Blum’s purple pigmented lichenoid dermatitis; Dukas-Kapetanakis eczematid purpura, itchy purpura; lichen golden, lichen purple.The difference between these pigment purples is mainly only in local features.

So, Shamberg’s disease can occur in children, middle-aged and old people. The lesion is represented by oval or indefinite outlines of yellowish-brown spots with small petechiae, similar in color to cayenne pepper. Frequent localization – on the lower extremities, especially on the ankles, but the rash can be on the buttocks, trunk. The rash turns brown, then disappears, but at the same time new petechiae appear.

Ring-shaped telangiectatic purpura Maiocchi is more often observed in adolescents, young people, especially women – spots 1-3 cm in diameter, often ring-shaped with punctate telangiectasias and petechiae of cayenne pepper color on the periphery. The rashes are asymptomatic, they exist for a long time.

Guzhero-Blum’s purpuric pigmentosa lichenoid dermatitis is a rare disease that occurs mainly in middle-aged and elderly men. The rash is similar to Schamberg’s disease, but it is predominantly composed of reddish-brown papules.It is extremely rare that the process may be accompanied by itching.

Eczematid-like purpura of Dukas-Kapetanakis occurs mainly in men on the lower extremities, is represented by purple spots, papules, plaques with signs of desquamation and an itching sensation.

Lichen aureus is a rather rare disease, usually represented by single pathological asymptomatic areas on the lower extremities, sometimes linear forms. The color of the elements is purple-brown with a golden hue.

Apparent intermediate mottled purpura of medium size include Waldenstrom hypergammaglobulinemic purpura, Gardner-Diamond syndrome, and Mondor disease.

Waldenstrom’s hypergammaglobulinemic purpura occurs mainly in women, clinically manifested by recurrent petechial large purple spots on the lower extremities (often with a burning sensation and slight itching). On examination, hypergammaglobulinemia, high titers of IgA and IgG of rheumatoid factor.Hypergammaglobulinemia can be either primary or secondary. In young patients, the process is predominantly primary, but over time there are signs of an autoimmune disease of the connective tissue, more often Sjogren’s syndrome, as well as rheumatoid arthritis and lupus erythematosus. Even transformation into lymphoma or multiple myeloma has been described.

Gardner-Diamond syndrome (psychogenic purpura) occurs mainly in women, while purpura is associated with psychiatric diseases (depression, uncontrollable aggressiveness and hostility, anxiety, hypochondria, masochism, sexual deviations, hysterical and borderline states, persecution mania).Painful, swollen areas of various sizes appear on any part of the body, disappear after 2 weeks, then relapse.

Mondor’s disease is characterized by superficial thrombophlebitis of the saphenous veins associated with erythema, ecchymosis, pruritus, arthralgia and even fever. The process, as a rule, is one-sided, mostly women are ill.

Platelet diseases deserve special attention, which include heparin necrosis (Fig.), Thrombocytosis secondary myeloproliferative diseases (reticular purpura), thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, cryoagglutination diseases, diseases of the organism caused by intravascular, opportunistic mushrooms).

It should be noted that heparin necrosis has become more frequent recently. Heparin necrosis (heparin-induced thrombocytopenia syndrome – the stage of platelet activation; heparin-induced thrombocytopenia occurs in patients with advanced thrombosis) is a rare but serious iatrogenic syndrome. Necrosis can occur after subcutaneous or intravenous administration of heparin, including consequences after minor use of heparin to maintain the infusion process.Heparin-induced thrombocytopenia occurs in 1-5% of adults receiving heparin therapy, and 30-90% of these patients develop thrombosis. Heparin necrosis is caused by antibodies not only to heparin, but also to the complex of heparin with platelet factor 4, while the protein appears on the surface of platelets, followed by cell aggregation. Blockage of blood vessels (veins, arteries, or cutaneous small vessels) leads to complications in the form of stroke, necrosis of fingers and extremities, or skin necrosis. Heparin necrosis manifests itself from the 5th to the 10th day of heparin therapy, however, it can develop much earlier in patients with hypersensitivity to heparin.In most patients with heparin-induced thrombocytopenia, the development of thrombosis has not been proven, and heparin necrosis can occur without thrombocytopenia. The lesions are predominantly slightly painful, clearly demarcated, non-inflammatory, typically purple or necrotic with a mesh pattern. Histologically, heparin necrosis is usually manifested by non-inflammatory blockage of blood vessels, including microvessels. It is quite difficult even for a pathologist to distinguish a white clot with a platelet plug from a red clot with a fibrin clot.

So, heparin necrosis belongs to the group of idiopathic progressive purpura, when platelet necrosis diseases are formed with cutaneous manifestations of blockage of small vessels. First of all, knowledge about the possibility of developing heparin necrosis is useful for both the family doctor and the therapist – one should be careful not to prescribe heparin for a long time to everyone.

Systemic coagulopathies with cutaneous manifestations include protein C and S disorders (congenital and acquired), congenital acute purpura with congenital disorder in the form of antithrombin III deficiency, warfarin necrosis, acute purpura with sepsis or post-infectious.Of particular note is warfarin necrosis, an acquired protein C dysfunction, which manifests itself 2-5 days after the initiation of warfarin administration. Lupus anticoagulant syndrome is caused by antiphospholipid antibodies and is associated with lupus erythematosus in one third of cases. By the way, this is what often reveals false positive reactions to syphilis with lupus. Skin manifestations of the syndrome – livedo reticular, leg ulcers, pseudovasculitis, gangrene of the fingers, cutaneous necrosis.

Finally, it is necessary to dwell on the diseases caused by embolism.These include cholesterol and oxalate embolism. Cholesterol embolism (warfarin blue toe syndrome) usually occurs in atherosclerotic patients. Fragmentation of the destroyed atheromatous plaque occurs. Three causes of embolism are noted: arterial and coronary catheterization; long-term prescription of anticoagulants; active thrombolytic therapy. Skin signs in most patients with cholesterol embolism are expressed in livedo reticular, multiple peripheral gangrene, cyanosis, ulceration, nodes, purpura.Therapy is mainly supportive, surgery is possible in the form of endarterectomy, taking aspirin, statins, antiplatelet drugs, corticosteroids. Warfarin necrosis should be taken into account in a timely manner by internists. Oxalate embolism is characterized by recurrent urolithiasis that has existed since childhood. Skin signs of systemic oxalosis – acrocyanosis, Raynaud’s phenomenon, peripheral gangrene, acral necrosis, livedo reticularis, erythematous ulcerating nodes, small calcifications.

As already noted, the following causes of purple are distinguished: syndrome of blockage of small vessels and vasculitis. Therefore, it is necessary to dwell on vasculitis of small vessels of the skin, which are also accompanied by rashes in the form of purple: leukocytoclastic vasculitis of the skin, anaphylactoid purpura, acute infantile hemorrhagic edema.

Anaphylactoid purpura (Schoenlein-Henoch purpura, rheumatoid purpura) predominantly occurs in children under 10 years of age after a previous respiratory infection.The obvious purpura, located on the lower limbs and buttocks, is accompanied by arthralgias and arthritis, abdominal pain, and in young children, melena. Renal vasculitis is often mild, but can become chronic.

Acute infantile hemorrhagic edema is predominantly observed in children aged 4 to 24 months, manifested by purple spots in the form of rings that may resemble a target. Favorite location – face, ears, lower limbs. The etiology is unknown, it is assumed a connection with infection, excessive drug load, immunization.

From the above material, it should be concluded that any appearance of purple is a sufficiently definite signal for the timely diagnosis of any of the listed diseases. Of course, purpura requires the consultation of a rheumatologist, nephrologist, hematologist. But, in addition to the significance of the problem of purple diseases, I would like to draw attention to the possibility of drug provocation of purple.

Medicna newspaper “Zdorovya Ukrainy 21 storichchya” No. 4 (473), lutiy 2020 p.

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