What are verrucous papules. How are vulvar verrucous papules diagnosed. What treatment options are available for vulvar verrucous papules. Who is most at risk for developing verrucous papules. Can verrucous papules be prevented. How do verrucous papules impact quality of life. When should someone seek medical attention for vulvar lesions.

Understanding Verrucous Papules: Characteristics and Appearance

Verrucous papules are small, wart-like growths that can develop on various parts of the body, including the vulvar region. These lesions typically appear as raised, flat-topped bumps on the skin surface. In the case study presented, the patient exhibited numerous 2- to 4-mm keratotic, flat-topped papules on her labia minora and majora.

The appearance of verrucous papules can vary, but they often share some common characteristics:

• Small size, usually 2-4 mm in diameter
• Raised above the skin surface
• Flat or slightly rounded top
• Rough, keratotic texture
• May appear in clusters or as individual lesions

Are verrucous papules always indicative of a specific condition. While verrucous papules can be associated with various underlying causes, their presence alone does not definitively diagnose a particular condition. A thorough clinical examination and additional diagnostic tests are often necessary to determine the exact etiology.

Vulvar Verrucous Papules: Causes and Risk Factors

Vulvar verrucous papules can develop due to various factors, and understanding these underlying causes is crucial for proper diagnosis and treatment. Some potential causes and risk factors include:

• Human papillomavirus (HPV) infection
• Chronic irritation or inflammation
• Lichen simplex chronicus
• Lichen sclerosus
• Hormonal changes
• Genetic predisposition

In the case study, the patient’s history of severe, erosive, macerated lesions in the intertriginous areas of her genitalia, occurring monthly around the time of her menses and worsening in the summer, suggests a potential hormonal or environmental influence on her condition.

Can certain lifestyle factors contribute to the development of verrucous papules. While not directly causative, certain lifestyle factors may increase the risk or exacerbate existing conditions. These may include poor hygiene practices, wearing tight or non-breathable clothing, and exposure to irritants or allergens.

Diagnostic Approaches for Vulvar Verrucous Papules

Accurate diagnosis of vulvar verrucous papules is essential for appropriate management. The diagnostic process typically involves several steps:

1. Thorough medical history
2. Physical examination
3. Dermoscopy
4. Biopsy and histopathological examination
5. Additional tests (e.g., PCR for HPV detection)

In the case study, a biopsy specimen of one of the verrucous papules was obtained for further analysis. This histopathological examination is crucial for confirming the diagnosis and ruling out other potential conditions.

How does a biopsy help in diagnosing verrucous papules. A biopsy provides detailed information about the cellular structure and composition of the lesion. This microscopic examination can reveal characteristic features of verrucous papules, such as epidermal hyperplasia, hyperkeratosis, and the presence of koilocytes in cases of HPV infection.

Clinical Presentation and Associated Symptoms

The clinical presentation of vulvar verrucous papules can vary among patients. In the case study, the 46-year-old African American woman presented with the following symptoms and signs:

• Numerous 2- to 4-mm keratotic, flat-topped papules on labia minora and majora
• Widespread hyperpigmentation
• Papillomatous plaques with mild crusting in intertriginous regions of anogenitalia
• Chronic, severe vulvar pruritus
• History of severe, erosive, macerated lesions in intertriginous areas

Do all patients with verrucous papules experience the same symptoms. No, the symptoms and clinical presentation can vary significantly between individuals. Some patients may be asymptomatic, while others may experience intense itching, discomfort, or pain. The severity and extent of lesions can also differ.

Treatment Options for Vulvar Verrucous Papules

The management of vulvar verrucous papules depends on the underlying cause, severity of symptoms, and individual patient factors. Treatment options may include:

1. Topical medications:
   • Corticosteroids for inflammation reduction
   • Imiquimod for immune response modulation
   • Retinoids for cell turnover regulation
2. Cryotherapy
3. Laser therapy
4. Electrocautery
5. Surgical excision
6. Photodynamic therapy

Which treatment option is most effective for vulvar verrucous papules. The most effective treatment varies depending on the individual case. Factors such as the underlying cause, extent of lesions, and patient preferences should be considered. A combination of treatments may be necessary for optimal results.

Differential Diagnosis: Conditions Mimicking Verrucous Papules

Several conditions can present with similar clinical features to verrucous papules, making differential diagnosis crucial. Some conditions to consider include:

• Condyloma acuminata
• Vulvar intraepithelial neoplasia (VIN)
• Molluscum contagiosum
• Seborrheic keratosis
• Vulvar squamous cell carcinoma
• Bowenoid papulosis

How can healthcare providers differentiate between verrucous papules and other similar-appearing lesions. Careful clinical examination, dermoscopy, and histopathological analysis are essential for accurate differentiation. Additionally, molecular testing for HPV and other potential pathogens may be necessary in some cases.

Impact of Verrucous Papules on Quality of Life

Vulvar verrucous papules can significantly affect a patient’s quality of life. The impact may include:

• Physical discomfort or pain
• Emotional distress and anxiety
• Sexual dysfunction
• Reduced self-esteem
• Social embarrassment

In the case study, the patient’s chronic, severe vulvar pruritus likely had a substantial impact on her daily life and overall well-being.

What strategies can help patients cope with the psychological impact of verrucous papules. Psychological support, patient education, and participation in support groups can be beneficial. Additionally, addressing any underlying psychological factors, such as stress or anxiety, may help improve overall outcomes.

Prevention and Long-term Management of Vulvar Verrucous Papules

While not all cases of verrucous papules can be prevented, certain measures may help reduce the risk or manage existing conditions:

1. Maintaining good genital hygiene
2. Wearing breathable, non-irritating underwear
3. Avoiding potential irritants or allergens
4. Regular gynecological check-ups
5. HPV vaccination (for HPV-related cases)
6. Stress management techniques
7. Proper management of underlying conditions (e.g., diabetes, autoimmune disorders)

Can lifestyle modifications alone prevent the recurrence of verrucous papules. While lifestyle modifications can help reduce the risk of recurrence, they may not be sufficient in all cases. Ongoing medical management and regular follow-ups with healthcare providers are often necessary for long-term control.

The Role of HPV in Verrucous Papules

Human papillomavirus (HPV) infection is a significant factor in the development of certain types of verrucous papules. Understanding the relationship between HPV and these lesions is crucial for both prevention and treatment:

• HPV types: Various HPV types can cause genital warts and verrucous papules, with types 6 and 11 being most common
• Transmission: HPV is typically transmitted through sexual contact
• Latency: The virus can remain dormant for extended periods before causing visible lesions
• Immune response: The body’s immune system plays a crucial role in controlling HPV infection and associated lesions

Does the presence of HPV-related verrucous papules increase the risk of cervical cancer. While most HPV types causing genital warts are low-risk for cancer, individuals with HPV infections should undergo regular cervical cancer screenings as recommended by their healthcare providers.

Histopathological Features of Verrucous Papules

The histopathological examination of verrucous papules reveals distinct features that aid in diagnosis and differentiation from other conditions. Key histological findings may include:

• Epidermal hyperplasia with acanthosis
• Hyperkeratosis and parakeratosis
• Papillomatosis
• Koilocytes (in HPV-related cases)
• Dilated blood vessels in the papillary dermis
• Inflammatory infiltrate in the dermis

How do these histological features help in determining the underlying cause of verrucous papules. The specific pattern and combination of histological features can provide valuable insights into the etiology of the lesions. For example, the presence of koilocytes strongly suggests an HPV-related origin, while certain inflammatory patterns may indicate other underlying conditions.

Emerging Treatments and Research Directions

The field of dermatology continues to advance, with new treatments and research directions emerging for the management of verrucous papules and related conditions:

1. Immunotherapies:
   • PD-1 inhibitors
   • Intralesional immunotherapies
2. Targeted therapies:
   • EGFR inhibitors
   • mTOR inhibitors
3. Novel topical formulations:
   • Nanoparticle-based drug delivery systems
   • Combination topical therapies
4. Gene therapies targeting HPV
5. Microbiome-based interventions

What potential benefits do these emerging treatments offer for patients with verrucous papules. These new approaches may provide more targeted, effective, and less invasive treatment options, potentially improving outcomes and reducing side effects compared to traditional therapies.

Special Considerations for Pregnant and Immunocompromised Patients

Managing verrucous papules in pregnant women and immunocompromised individuals requires special considerations:

Pregnant patients:

• Limited treatment options due to potential fetal risks
• Increased risk of lesion growth and spread during pregnancy
• Potential for vertical transmission of HPV to the newborn

Immunocompromised patients:

• Higher risk of developing extensive or persistent lesions
• Increased likelihood of HPV-related complications
• Potential for atypical presentations of verrucous papules

How should treatment approaches be modified for these patient populations. Treatment plans should be tailored to balance efficacy and safety, often requiring close collaboration between dermatologists, obstetricians, and other specialists. Conservative approaches and careful monitoring may be necessary, particularly during pregnancy.

The Importance of Patient Education and Follow-up

Effective management of vulvar verrucous papules extends beyond medical interventions. Patient education and regular follow-up play crucial roles in achieving optimal outcomes:

1. Patient education topics:
   • Nature of the condition and its potential causes
   • Importance of treatment adherence
   • Self-examination techniques
   • Lifestyle modifications to reduce risk factors
2. Follow-up considerations:
   • Regular monitoring of treatment response
   • Screening for potential complications or recurrence
   • Adjustment of treatment plans as needed
   • Addressing ongoing patient concerns and questions

How can healthcare providers ensure effective patient education and follow-up. Implementing a multidisciplinary approach, utilizing patient education materials, and establishing clear communication channels can enhance patient understanding and adherence to treatment plans. Regular follow-up appointments and telemedicine options can facilitate ongoing monitoring and support.

Vulvar Verrucous Papules | Dermatology | JAMA Dermatology

Vulvar Verrucous Papules | Dermatology | JAMA Dermatology | JAMA Network










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Off-Center Fold

February 1999

George J. Murakawa, MD, PhD^{; Russell Kerschmann, MD; Timothy Berger, MD}

Author Affiliations

LORILOWEMD

Arch Dermatol. 1999;135(2):203-b-208. doi:10-1001/pubs.Arch Dermatol.-ISSN-0003-987x-135-2-dof0299

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A 46-year-old African American woman was referred from the gynecology service for evaluation and treatment of verrucous papules on her vulva. Her history was remarkable for a 15- to 20-year history of severe, erosive, macerated lesions in the intertriginous areas of her genitalia that occurred monthly around the time of her menses and worsened in the summer. She also complained of chronic, severe vulvar pruritis.

On examination, the patient had numerous 2- to 4-mm keratotic, flat-topped papules on her labia minora and majora (Figure 1). She also had widespread hyperpigmentation and papillomatous plaques with mild crusting in the intertriginous regions of her anogenitalia. A biopsy specimen of one of her verrucous papules was obtained (Figure 2 and Figure 3).

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Verrucous Cobblestonelike Papules and Nodules of the Right Lower Limb

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An obese 58-year-old man was admitted to the cardiology department for poorly controlled congestive heart failure. He was referred to the dermatology department with progressive painless swelling of the right lower limb of a year’s duration. He had chronic right lower limb insufficiency of 3 years’ duration with a history of a recurrent right medial malleolus ulcer and cellulitis. There was no notable travel or family history. Physical examination revealed woody edema of the right lower limb with verrucous cobblestonelike papules and nodules, foul-smelling odor, and thick crusts that were easily removed.

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The Diagnosis: Elephantiasis Nostras Verrucosa

Elephantiasis nostras verrucosa (ENV) is a chronic deforming disorder characterized by hyperkeratosis and papillomatosis of the epidermis with underlying woody fibrosis of the dermis and the subcutaneous tissue in the setting of chronic nonfilarial lymphedema. Mossy papules, plaques, and cobblestonelike nodules are classic clinical features of ENV (Figure). The cobblestone lesion often is foul smelling and may be colonized with multiple bacteria and fungi.¹

Verrucous cobblestonelike papules and nodules on the right lower limb.

The differential diagnosis includes filariasis, chromoblastomycosis, and venous stasis dermatitis. Filariasis is infection with the filarial worms Wuchereria bancrofti, Brugia malayi, or Brugia timori. These parasites are transmitted to humans through the bite of an infected mosquito and develop into adult worms in the lymphatic vessels, causing lymphedema. Elephantiasis, the classic sign of late-stage disease, also causes swelling of the legs and genital organs that can be disfiguring. Thus travel history to Southeast Asia and Africa regions is particularly important.

Chromoblastomycosis is a chronic fungal infection of the skin and subcutaneous tissue caused by traumatic inoculation of a specific group of dematiaceous fungi, usually Fonsecaea pedrosoi, Phialophora verrucosa, Cladosporium carrionii, or Fonsecaea compacta. Chromoblastomycosis-induced lymphedema may mimic elephantiasis. Thus it is important to perform fungal culture and fungal scraping, which will show typical thick-walled, cigar-colored, sclerotic cells (known as Medlar bodies).

The skin changes of venous stasis dermatitis include edema, varicosities, hyperpigmentation, atrophie blanche, diffuse red-brown discoloration representing deep dermal deposits of hemosiderin, and lipodermatosclerosis. Venous stasis dermatitis is caused by chronic venous insufficiency. It is one of the predispositions to ENV, as it can cause repeated infections and cellulitis leading to blockage of lymphatic drainage and secondary lymphedema. A study of 21 cases highlighted that chronic venous insufficiency may be an underappreciated risk factor in the pathogenesis of ENV.²

The development of verrucous cobblestonelike plaques in ENV occurs with chronic lymphedema. Chronic lymphedema can be caused by a variety of etiologies including infection, chronic venous stasis, congestive heart failure, obesity, trauma, tumor obstruction, and radiation. Our patient was obese with chronic venous stasis, congestive heart failure, and recurrent cellulitis that can lead to secondary lymphatic obstruction and edema, all contributing to the picture of ENV. Because of the unilateral presentation in our patient, we believe that recurrent infections and inflammation are the important risk factors that lead to fibrosis of the dermis and lymph channels, ultimately resulting in ENV.

The diagnosis of ENV is based on the patient’s history and characteristic skin changes. However, a biopsy should be performed if the diagnosis is not clinically apparent, if the lesion looks suspicious for malignancy, or if areas of chronic ulceration exist. Histologic findings include hyperkeratosis as well as a collarette of acanthotic epidermis extending around dilated lymph vessels in the upper dermis. Sometimes a few dilated lymph vessels present in the deeper or subcutaneous tissue. Bacterial and fungal culture should be done to exclude concomitant infection. Ultrasonography can be used to evaluate the lymphatic and venous systems.

Treatment of ENV aims at improving lymphedema and preventing infection. Physical therapy to improve lymphedema includes manual lymphatic drainage, massage, compression stockings, elastic bandages, and leg elevation. In cases associated with obesity, weight loss is recommended. In cases of recurrent cellulitis or lymphangitis, long-term antibiotic therapy with agents such as penicillins or cephalosporins is sometimes used.³ Success has been reported with the use of oral and topical retinoids, such as acitretin and tazarotene, which are thought to decrease epidermal proliferation, fibrogenesis, and inflammation.^4,5 Our patient was started on tretinoin cream. Surgery is reserved for cases of lymphedema resistant to medical therapies, including debridement,⁶ lymphatic and lymphovenous anastomosis,⁷ lymphatic transplantation, and amputation for severe cases.

Malignancies can develop in areas of chronic lymphedema such as lymphangiosarcoma, squamous cell carcinoma, Kaposi sarcoma, B-cell lymphoma, and malignant fibrous histiocytoma. The morbidity and mortality usually are from the underlying medical problems rather than ENV itself. Our patient died 1 month after the diagnosis of ENV from his poorly controlled heart failure.

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Clinical features of Kirle’s disease

Follicular and parafollicular hyperkeratosis penetrating the skin (Kirle’s disease) is a rare genetically determined disease characterized by impaired keratinization. The disease is included in the group of perforating dermatoses, which also includes perforating folliculitis, serpiginous perforating elastosis, and reactive perforating collagenoses [1]. It was first described by J. Kyrle [2] in 1916, who observed dermatosis in a patient with diabetes mellitus. The etiology of Kirle’s disease has not been sufficiently studied to date, presumably autosomal recessive inheritance. Family cases are given, the relationship of the disease with liver damage and the development of secondary vitamin A deficiency, with vasculopathy, chronic renal failure, diabetes mellitus, liver and spleen diseases, congestive heart failure, hyperlipidemia, hypothyroidism is indicated. At the same time, there is evidence in the literature that the disease can develop in the absence of severe concomitant somatic pathology.

The pathogenesis of Kirle’s disease also remains poorly understood. With this dermatosis, desynchronization of the processes of cell division and maturation is noted due to an increase in the rate of differentiation of keratinocytes, which exceeds the rate of cell proliferation. Therefore, the parakeratotic cone partially penetrates deeper, into the disturbed epidermis, and causes its perforation into the dermis. The result of this is the formation of horny and parakeratotic plugs [3, 4]. Keratinization begins at the border of the epidermis and dermis. According to another point of view, skin trauma can serve as a trigger. Nevertheless, the molecular and cellular basis of the Koebner phenomenon in this case, as well as in other dermatoses, still remain unclear [5].

There is no single classification of follicular keratoses. There are papular, atrophic and vegetative follicular keratoses [6].

Kirle’s disease occurs more often in adults in their third or fourth decade of life, but cases have also been described in children. It is believed that women get sick more often than men.

We observed a 44-year-old man who, 2 months before the examination, had papular-erosive elements at the site of scratching and periodically suffered from mild itching in the lesion (Fig. 1). Initially, with this dermatosis, itchy follicular or parafollicular papules appear: first the color of healthy skin, then red-brown in color, 1-3 to 10 mm in diameter, which are located symmetrically on the back of the hands and feet (Fig. 2). Then the process spreads to the extensor surface of the legs and forearms (Fig. 3), less often the upper parts of the body (Fig. 4), head; isolated cases of lesions of the mucous membrane of the mouth and palms are described. The onset of the disease is gradual, new rashes appear as the old ones disappear. The center of the elements is keratinized, along the periphery of which there are no inflammatory phenomena. The rash tends to grow peripherally and coalesce to form dry warty plaques. Their deep penetration into the dermis with an inflammatory-granulomatous perifocal reaction is noted (Fig. 5). In places of combing, the elements have a linear arrangement (Kebner phenomenon) (Fig. 6). After removing the horny masses, slightly moist or bleeding crater-like depressions are exposed (Fig. 7, 8). The course of the disease is chronic, individual efflorescences persist for up to 6-8 weeks and regress, leaving smooth scars or hyperpigmentation (see Fig. 1).

Rice. 1. Follicular papules, excoriations, secondary hyperpigmentation in a patient with Kirle’s disease.

Rice. 2. Follicular papules on the back of the hands.

Rice. 3. Process on the extensor surface of the forearm.

Rice. 4. Process with localization on the skin of the upper parts of the trunk and shoulder.

Rice. 5. Grouped location of the rash, with the formation of a dry plaque on the skin of the right thigh.

Rice. 6. Linear arrangement of elements (Kebner phenomenon) on the skin of the lower parts of the body.

Rice. 7. Crater-shaped depressions on the surface of rashes on the skin of the forearm.

Rice. 8. Crater-like depressions on the surface of rashes on the lower leg.

Histological examination of Kirle’s disease is characterized by the formation of invaginations in the epidermis, filled with horny masses. The epidermal invagination contains a well-developed granular layer, with the exception of the contact zone of the parakeratotic cells of the horny cone with the epidermis. Inside the horny plugs, focal parakeratosis is observed and basophilic stained masses are found. Under the horny plugs, the epidermis can become thinner to 2-3 layers of cells; in the adjacent areas, on the contrary, it is acanthotically thickened. In long-term elements, keratotic masses penetrate the dermis, and a granulomatous reaction is formed around the penetration sites from lymphocytes, histiocytes, epithelioid and giant multinucleated cells. There is a death of the sebaceous glands in the area of ​​the affected follicles. Collagen (but not elastic) fibers show signs of dystrophy. In the hypodermis, edema, infiltration, accumulation of acidic glycosaminoglycans are detected. Apparently, the rate of differentiation and keratinization, which contributes to the formation of dyskeratotic foci, exceeds the rate of cell proliferation. Therefore, the parakeratotic cone partially penetrates deeper, into the disturbed epidermis, and causes its perforation into the dermis.

Basic principles of differential diagnosis in Kirle’s disease are formulated in the classic monograph by B.A. Berenbein, A.A. Studnitsyn (1989) and in the article by A.N. Lvova et al. (2011) [6, 7]. A distinction should be made with Miescher-Lutz serpiginous perforating elastosis, follicular form of lichen planus, Devergy’s disease, Darier’s disease, Mibelli’s porokeratosis, Flegel’s persistent lenticular keratosis, multiple keratoacanthoma.

The greatest difficulties arise in the differential diagnosis of Kirle’s disease and serpiginous perforating Miescher-Lutz elastosis. An important distinguishing feature in Kirle’s disease is the localization of follicular keratotic eruptions on the legs, and in serpiginous perforating elastosis – on the neck, nape, elbows. With elastosis, single polycyclic serpiginating foci with a slightly sunken somewhat atrophic center are observed, while with Kirle’s disease, rashes are usually multiple, with massive hyperkeratotic plugs, in addition to horny papules, pigmented scars are often found. It should be taken into account that serpiginous perforating elastosis usually begins at a young age [7].

Differential diagnosis with the follicular form of lichen planus is based on the fact that in Kirle’s disease the rashes have a much more pronounced hyperkeratotic character and sharper borders, more often yellowish-brown, and not lilac, as in lichen planus, conical rather than flattened shape. In Kirle disease, pigmented scars are also found, and lichen planus often manifests on the mucous membranes, palms or soles (rash-free areas in Kirle disease), and typical polygonal papules with an umbilical depression in the center can also be found. In unclear cases, a histological examination is necessary to differentiate these conditions.

With pityriasis versicolor pilaris, horny papules are smaller, brighter reddish-yellow in color, characteristic cone-shaped scales on the surface, significantly pronounced on the back surface of the hands, which are not warty in nature and tend to spread, merge, form diffuse foci on skin of the face, scalp and other parts of the body up to the development of erythroderma. A feeling of tightening of the skin and itching can often be observed. Devergie’s disease is also characterized by hyperkeratosis of the palms and soles [6, 7].

Darier’s disease differs from Kirle’s disease in localization characteristic of seborrheic processes (scalp, face, neck, interscapular region). Papules in Darier’s disease are smaller, tend to merge, especially in the folds of the skin with the formation of vegetative foci and linear erosions. On the back of the hands, papules in Darier’s disease resemble flat warts, there are no pigmented scars. The histological picture in Darier’s disease is characterized by the presence of dyskeratosis with the formation of round bodies and grains, the formation of suprabasal slit-like cavities in the epidermis [6, 7].

In contrast to Kirle’s disease in Mibelli’s porokeratosis, papules are smaller, similar to comedones, localized not only on the shins, but also on the trunk, face, and genitals. Their characteristic feature is not the verrucous layering of horny masses in the form of a cone, as in Kirle’s disease, but the presence of a depression surrounded by a hyperkeratotic ridge. This feature of the papules is also preserved when they merge into closed polycyclic figures, the central part of which may be slightly atrophic, filled with hyperkeratotic masses, and the peripheral part may be raised, in the form of a horny comb [6].

Flegel’s lenticular persistent hyperkeratosis, like Kirle’s disease, is localized mainly on the feet and legs. However, unlike Kirle’s disease, in this disease the papules are more persistent (they exist for many years), smaller in size, without a clear confinement to the hair follicles, and are not prone to merging. In the center of the nodules there is a slight retraction, and their surface is covered with scales, which gives the papules some resemblance to psoriatic elements. Histologically, Flegel’s dermatosis is characterized by the presence of a large number of capillaries in the dermis, surrounded by an infiltrate, which in some places can acquire a strip-like configuration [6].

Multiple keratoacanthoma, in contrast to Kirle’s disease, occurs more often in open areas of the skin. The color of the tumor with kerato-acanthoma is either flesh-colored or more saturated pink. Of great diagnostic importance is the fact of rapid development and regression of the tumor in keratoacanthoma compared with rashes in Kirle disease, which, although they regress, may exist for months or even years before [7].

Vitamins (A, C, B) are recommended for the treatment of Kirle’s disease, and aromatic retinoids (neotigazon, tigazon, etretinate, acitretin) are recommended for persistent course [8]. So, A.N. Lvov [7] prescribed acitretin at a dose of 25 to 50 mg per day, daily or every other day, and noted a pronounced positive effect with good tolerance. Softening creams are applied externally, and topical glucocorticosteroid agents of medium strength, sulfur-salicylic ointments, ointments with urea, topical retinoids are applied to the most infiltrated elements. After removal of horny layers, it is possible to carry out ultraviolet irradiation, electrocoagulation of individual foci [8].

This observation will allow dermatovenereologists and cosmetologists to focus on the clinical features of this rare skin pathology, timely make a clinical diagnosis and conduct adequate therapy.

No conflict of interest.

differential diagnosis and efficacy of patient therapy with isotretinoin

Follicular and parafollicular hyperkeratosis penetrating into the skin (Kirle’s disease; CD), described by the Austrian dermatologist and pathologist Josef Kirle in 1916 [1]. Kirle observed skin changes characteristic of this dermatosis in a patient with diabetes mellitus and singled out the disease as a separate nosological form [1, 2]. Cases of CD without severe concomitant pathology, as well as a combination of this dermatosis with diabetes mellitus, functional disorders of the liver, kidneys (including against the background of malformations of the ureters and renal pelvis) are described [3, 4].

The etiology of the disease remains unknown. Most dermatologists believe that the acceleration of keratinization and differentiation of epidermal cells plays a leading role in the pathogenesis of Kirle’s disease. This process leads to the formation of keratotic “plugs” with areas of parakeratosis, which penetrate the epidermis and dermis and cause a chronic inflammatory reaction of the adjacent dermal tissues, similar to a reaction to a foreign body [2]. The phenomenon of transepidermal elimination, characteristic of CD and other perforating dermatoses, is not the cause, but the end result of accelerated keratinization of epidermal cells.

The disease is extremely rare. There are few descriptions of clinical cases of CD in Russia. In each of them, clinical manifestations characteristic of dermatosis are noted and the features of each case are emphasized. Thus, a combination of CD with cholelithiasis, chronic pyelonephritis, a case of CD in a 1.7-year-old child, as well as observations of patients with this dermatosis without concomitant pathology have been described [4–7]. Kirle disease is more common in patients older than 30 years, but can also begin in childhood [8]. A characteristic eruptive element is a dense nodule 3–8 mm in diameter with a horny “plug” in the center. Rashes are localized on the skin of the extensor surface of the limbs, trunk, occasionally on the head. The elements of the rash are disseminated, in some places grouped with a tendency to merge with the formation of separate reddish-brown rounded plaques with a warty surface. During mechanical removal of the horny cone in a separate typical papule, a crater-like deepened erythematous surface is exposed, on the walls of which bloody discharge is visible. Subsequently, this defect heals with the formation of a hyperpigmented scar [1]. An ichthyosiform skin change is also possible [8]. The course of the disease is long, chronic, monotonous, complete recovery is doubtful [9].

We present a description of a middle-aged patient who was diagnosed with hyperkeratosis follicular and parafollicular, perforating into the skin, 13 years after the onset of the disease. The patient was presented and discussed at the meeting No. 1048 of the St. Petersburg Society of Dermatovenereologists. V.M. Tarnowski January 29, 2019

Patient D. , aged 52, first applied to the outpatient department of the clinic of dermatovenereology of the St. Petersburg State Medical University named after. acad. I.P. Pavlova in April 2018 with complaints of widespread rashes, hair loss in the affected areas and decreased sweating. The patient was born in the village of Zabituy, Irkutsk region; at the age of 1, he moved to Angarsk with his parents. He grew and developed according to his age. Hereditary diseases are denied. Occupational hazards: from 1986 to 1999 worked as an engineer in a chemical production (contact with halogens). She has been smoking since the age of 16 and rarely drinks alcohol. Allergic reactions are denied.

He considers himself ill since 2006 (from the age of 39), when for the first time painless dense nodules with a depression and a black horny cork in the center began to appear on the anterior surface of the abdomen, which the patient tried to squeeze out on his own. In those cases when it was possible to squeeze out a dark-colored cork, in addition to it, a thick grayish-white mass stood out. The rash slowly spread to the neck, trunk, limbs and face. In the abdomen and right shin, the elements merged, forming dense foci. Scars formed after the regression of individual small papules. In the same areas, vellus hair loss was noted. Sweating also gradually decreased. The rash spread slowly, but over the past 2 years, the disease began to progress more intensively. The patient repeatedly applied to dermatologists at the place of residence in Angarsk. According to the patient, the diagnosis was not established, he did not receive treatment. He was sent to Irkutsk for an additional examination, where in January 2018 a biopsy of the affected skin was performed. Conclusion: microscopic picture of scleroderma. It is recommended to exclude collagenosis, hair follicle disease, Kirle disease, Flegel’s keratosis, ichthyosis, Darier’s dyskeratosis.

To verify the diagnosis, the patient in April 2018 applied for a consultation at the outpatient department of the clinic of dermatovenereology of the St. Petersburg State Medical University named after. acad. I.P. Pavlova. The man was examined and discussed by professors and associate professors of the department with the clinic. Given the clinical manifestations, the specialists suspected a disease from the group of perforating dermatoses, recommended a second skin biopsy, inpatient examination and treatment.

In June 2018, the patient was hospitalized in the clinic of dermatovenereology, St. Petersburg State Medical University named after. acad. I.P. Pavlova. Upon admission to the clinic, the general condition is satisfactory, consciousness is clear. The physique is correct, the constitution is normosthenic. Height 184 cm, weight 96 kg, body mass index 28 kg/m ² . Subcutaneous fat is moderately developed, evenly distributed. The thyroid gland is not visually determined, painless on palpation, soft elastic consistency, mobile when swallowing. Peripheral lymph nodes are not enlarged, painless on palpation. The musculoskeletal system without visible changes. Heart sounds are clear, clear, no murmurs. Pulse 72 beats / min, rhythmic, symmetrical, satisfactory properties, blood pressure 130/90 mmHg Breathing is hard, not weakened. Respiration rate 16 per minute. The abdomen is not enlarged, not swollen, soft on palpation, except for the lesion on the anterior surface of the abdominal wall, painless. The liver is not enlarged, does not protrude from under the edge of the costal arch. The kidneys and spleen are not palpated. Tapping on the lower back in the projection of the kidneys is painless on both sides. Physiological departures are normal (from the words).

Unaffected skin is white with a pinkish tint. The skin lesion is widespread (Fig. 1), Fig. 1. Damage to the skin of the abdomen and face. rashes are located mainly symmetrically on the face, trunk, limbs, with the exception of a confluent focus in the region of the right lower leg. The skin of the face is red, dry, with numerous milia-like elements and separate comedo-like papules in the temporal areas, mainly on the right. On the neck, trunk, and extremities, eruptive elements are represented by numerous papules 2–8 mm in diameter with umbilical depressions and a black horny plug in them. Individual papules are larger, slightly hyperemic. In addition to these elements, on the extensor surface of the shoulders (Fig. 2) Fig. 2. Comedonous papules on the skin of the right shoulder. and back (in the projection of the shoulder blades) there are small cone-shaped flesh-colored papules. When stroking these areas, the “grater” symptom is determined. On the anterior surface of the abdomen there is a bluish lesion 20 × 30 cm with an uneven, bumpy surface, consisting of clustered bluish-brown dense, painless nodes, on the surface of which numerous black horny plugs 2-3 mm in diameter are visible, located below the level of the surrounding skin (Fig. 3). Rice. 3. A large lesion on the skin of the abdomen. In addition, sunken atrophic scars with a diameter of 3 to 8 mm are visible within the focus. With strong squeezing of papules with black horny plugs, with great difficulty it was possible to squeeze out a significant amount of a thick grayish-white mass with separate small lumps. On the lateral surface of the right leg, there is a round bluish-red focus with clear boundaries 6 cm in diameter, protruding above the skin surface with a perifocal zone of inflammation of a stagnant red color, about 5 mm wide. In the center of the focus, an area of ​​atrophy with a diameter of about 5 cm of a lighter color is determined (Fig. 4). Rice. 4. Plaque with atrophy in the center. On the extensor surface of the middle third of the left forearm, there is a similar oval focus 2×3 cm in size, dense, without atrophy. On palpation, all eruptive elements are painless.

The results of clinical and biochemical blood tests are normal. Conclusion of the endocrinologist dated April 17, 2018: data for endocrine pathology were not revealed. The result of ultrasound of the abdominal organs and kidneys dated April 17, 2018: moderate diffuse changes in the pancreas. The result of ultrasound of the thyroid gland dated February 5, 2018: no echostructural pathology was detected.

On April 19, 2018, the patient underwent a skin biopsy in three lesions: the skin of the right shoulder (comedo-like papules), the abdomen (deep node biopsy), and the right shin (plaque marginal zone). The revealed histological changes were of the same type. The most pronounced and most characteristic pathomorphological changes were found in preparations from a focus on the skin of the lower leg: diffuse and pronounced focal hyperkeratosis, filling invaginations of different depths, some of them penetrate the epidermis and dermis and reach the subcutaneous fat. Along the edges of the corneal plug, there are single foci of parakeratosis. Loose masses in the intussusceptions, some of them stained basophilically, the other part – eosinophilically (Fig. 5, 6). Rice. 5. Pathological changes (staining with hematoxylin and eosin; ×80): 1 – invagination of the epidermis, penetrating into the dermis; 2 – horny masses, partly basophilic, partly eosinophilic; 3 — polymorphic cellular infiltrate around the invagination. Fig. 3. 6. Pathological changes (staining with hematoxylin and eosin; ×80). 1 – invagination of the epidermis with horny masses; 2 — polymorphic cellular infiltrate under invagination; 3 – the muscle that lifts the hair. The epidermis in the zone of invaginations with areas of atrophy, its integrity is not broken. Around the intussusceptions, there is a polymorphic cellular infiltrate, which is most pronounced under them, in the lower parts of the dermis and in the subcutaneous fat. The infiltrate is predominantly lymphocytic-histiocytic with giant cells of the Pirogov-Langhans type (see Fig. 6, 7). Rice. 7. Pathological changes, fragment of fig. 6 (staining with hematoxylin and eosin; ×80). 1 – a section of the muscle that lifts the hair; 2 – giant cells of the Pirogov-Langhans type. There is also a slight perivascular infiltration. The muscles that raise the hair are oriented obliquely towards the invaginations and infiltrates around them (see Fig. 6). In a large number of serial sections, hair and sebaceous glands were not detected (neither in the area of ​​the described changes, nor in the areas next to them). Isolated sweat glands were found. The absence of hair and sebaceous glands in the preparations as reliable signs of hair follicles does not allow us to accurately determine whether the intussusceptions are follicular. The location of the levator pilus muscle in relation to the invagination and infiltrate suggests a parafollicular location of the invaginations. Weigert staining revealed no pathological changes in elastic fibers. Conclusion: the described changes do not contradict the clinical diagnosis of follicular and parafollicular hyperkeratosis, perforating into the skin (BC).

Currently, not all authors recognize the follicular localization of lesions in CD [10, 11]. We previously diagnosed widespread rashes in the presented patient as manifestations of follicular and parafollicular hyperkeratosis penetrating into the skin (BC).

Differential diagnosis was performed with persistent lenticular keratosis (Flegel’s disease; H. Flegel, 1958), intrapapillary perforating elastosis (J. Miescher, 1955) or follicular serpiginating keratosis (W. Lutz, 1953 g.), acne vulgaris and inverse, follicular dyskeratosis (Dariaer’s disease; morbus Darier ), pityriasis rubra pilaris (Devergy’s disease; pityriasis rubra pilaris ), follicular form of lichen planus (lichen ruber follicular is, syn. lichen ruber planopilaris) , multiple keratoacanthoma and disseminated superficial actinic porokeratosis.

With similar localizations of persistent lenticular hyperkeratosis (HLS; Flegel’s disease, H. Flegel, 1958) differ from the rashes in Kirle’s disease. With GLS, these are red-brown dense nodules 1–5 mm in diameter with a horny scale on the surface. When they are scraped, unlike BC, hyperkeratotic lamellar scales are easily removed from the surface, without leaving a deep crater-like defect. The hard-to-remove keratotic plug characteristic of CD and pigmented scars are absent. Hyperkeratotic papules in HLS are smaller, not prone to fusion. Some papules, slowly increasing, acquire a polygonal shape, which is not typical for B.K. On histological examination, there is no massive hyperkeratotic plug and epidermal invagination, there is no phenomenon of epidermis and dermis perforation characteristic of CD. In the dermis there is lymphocytic infiltration, there is no granulomatous inflammation, the papillae of the dermis are penetrated by numerous capillaries.

In contrast to CD, serpiginous perforating elastosis (ESP) ( syn.: keratosis follicular serpiginous Lutz, 1953) is a hereditary disease and develops in adolescence. In 40-50% of cases, it is combined with other congenital connective tissue diseases: Ehlers-Danlos syndrome, elastic pseudoxanthoma, Morfan’s syndrome, Down’s syndrome and others. Rashes are localized on the neck, back of the head, less often in the area of ​​the elbow and knee joints, which is not typical for B.K. Rashes in serpiginous perforating elastosis are represented by dark brown conical papules 2–5 mm in diameter with small horny plugs on the surface. When scraping, horny plugs are easily removed from the surface of the nodules, unlike BC, leaving small depressions that heal with small scars. An important distinguishing feature of lesions in ESP is their grouping and merging into separate plaques in the form of arcs or rings, their peripheral growth with the formation of a separate serpiginous focus with polycyclic outlines. The partially regressed center of this focus is atrophic and somewhat sunken. Histological examination (with fuchsilin staining according to Weigert to identify elastic fibers) with ESP reveals an increase in the number and thickness of elastic fibers in the upper part of the dermis. In contrast to CD, ESP involves a transepidermal displacement from bottom to top of amorphous dermal material, stained like elastic fibers. Hyperkeratotic cone is slightly expressed. In the dermis, granulomatous inflammatory infiltration.

In the clinical case presented by us, rashes in some areas resembled non-inflammatory papular “open” acne ( acne comedonica ), which are characteristic of acne vulgaris and also occur in conglobate and inverse acne. It is known that acne vulgaris and conglobata, as a rule, occur in boys and men of young age. The rashes are located in the seborrheic zones (face, chest, back), are always associated with sebaceous hair follicles and are accompanied by hyperplasia and hyperfunction of the sebaceous glands, which is not typical for B. K. In addition, in these areas, in addition to “open” comedones, inflammatory elements develop (acne papulosa, acne pustulosa, acne abscedens et conglobata ), which does not happen with B.K. Comedones in acne vulgaris can be easily squeezed out, while massive horny plugs in CD can be squeezed out with difficulty, and in this place there remains a deep crater-like defect that heals with a scar. With inverse acne, which develops mainly in men, manifestations are localized in large folds – axillary, inguinal-femoral, intergluteal, as well as in the genital area, on the buttocks. Rashes (intertriginous abscessing nodes, abscessing perifolliculitis, bridge-like melting infiltrates, anastomosing fistulas) with a density of the board often affect areas atypical for acne, proceed for a long time, tend to increase in the size of the lesion, are periodically accompanied by exacerbations with an increase in temperature. Characteristic tetra acnede (acne conglobata, acne inversa, folliculitis nuchae abscedens et suffodiens, sinus pilonidalis ) is absent in B. K. Histological examination ( acne comedonica ) shows a sebaceous-horn plug located at the mouth of the hair follicle, hyperplastic sebaceous glands and minor inflammatory infiltrates around the sebaceous gland and hair follicle are visible, which is not typical for CD.

Rare follicular lichen planus (PLL; lichen ruber follicularis , lichen ruber planopilaris , lichen ruber acuminatis ) may clinically resemble rashes in Devergie and B.K. A characteristic element of the FCPL rash is a small perifollicular cone-shaped papule with a horny spine in the center. Unlike BC, in a cone-shaped and smaller papule, the horny spine is also small and regresses without a trace (without a scar). Such grouping elements are located mainly on the extensor surfaces of the limbs and the scalp, which leads to the development of cicatricial focal alopecia. With damage to the armpits and the pubic region, persistent hair loss develops without clinical manifestations of skin atrophy (limited damage and death of hair follicles), which also does not happen with B. K. In FCPL, rashes on smooth skin usually do not form large plaques and regress on their own within a few months, which is not typical for CD. In some cases, small follicular papules are combined with individual rash elements characteristic of typical lichen planus – flat polygonal red-violet papules with a smooth shiny surface and an umbilical depression in the center, which does not happen with B.K. On histological examination of FCLP lesions, the corneal plug in the dilated hair follicle is much smaller than in CD and penetrates shallowly into the epidermis (there is no phenomenon of perforation of the epidermis and dermis characteristic of CD). In the lower part of the hair follicle there is a dense, predominantly lymphocytic infiltrate (non-granulomatous infiltrate, as in CD), leading to obliteration of the hair and death of the hair follicle.

The manifestations of CD differ from the manifestations of superficial actinic porokeratosis (SPA) by the localization of the lesions, their appearance and the result of their evolution. PPA elements are located on open areas of the body (forearms, shins, face) and initially represent numerous small brownish-gray papules with a horny spine in the center; gradually increase in size and merge into characteristic superficial, weakly infiltrated ring-shaped foci up to 0.5-1 cm in diameter. They are pinkish or yellowish-brown in color, surrounded by a thin horny roller, which is hardly noticeable, but easily determined by palpation. In the center, the foci sunk somewhat, slight atrophy of the skin is noticeable. There are no massive horny plugs characteristic of CD, there are no scars after regression of rashes. The histological picture of these dermatoses also differs. With PPA, in the central part of the element, keratin invagination into the epidermis is visible in the form of a cone, which is a parakeratotic column (horny plate). There is nonspecific lymphocytic infiltration in the dermis, which is not typical for CD.

Follicular dyskeratosis (Darier’s disease, morbus Darier ), unlike CD, usually occurs in childhood or adolescence. Rashes are located mainly on seborrheic areas (chest, back, face) and on the extensor surfaces of the limbs. Brown flat hyperkeratotic papules are smaller than in CD (diameter 2-3 mm), covered with a horny scale, which is tightly bonded to the surface. The elements are located predominantly follicular, tend to merge and exist for a long time. In some cases with Darier’s disease, in addition to hyperkeratotic papules, vesiculo-bullous elements appear, linear erosions and vegetations may develop in the folds, which does not happen with B.K. In addition, Darier’s disease is characterized by ruptures of papillary lines on the fingertips and degenerative changes in the nail plates, which is also uncharacteristic for B.K. Histological examination in Darier’s disease reveals that, unlike CD, follicular hyperkeratosis does not penetrate deep into the epidermis and dermis, is accompanied by dyskeratosis with the formation of “round bodies” and “grains” in the prickly and stratum corneum and focal suprabasal acantholysis with the formation of lacunae.

Pityriasis pilaris pilaris ( pityriasis rubra pilaris , Devergie’s disease – BD) and CD can occur in both childhood and adulthood. The primary element of the rash in BD is a small (1 mm in diameter) red perifollicular papule with a pointed horny spine in the center. With the progression of BD, unaffected interfollicular spaces become hyperemic and confluent erythema develops with pityriasis peeling, which covers entire areas on the trunk and extremities. These confluent red-orange (salmon) lesions, where the original small perifollicular papules cannot be identified, have small (1 cm in diameter), well-defined and slightly sunken rounded areas of white, apparently unchanged skin. The process consistently spreads from the head to the lower extremities (top to bottom). All this is significantly different from the manifestations of B.K. With B.D. perifollicular papules with a horny spine in the center are clearly visible on the back of the phalanges of the fingers, knees and elbows. For B.D. palmoplantar keratoderma and dystrophic changes in the nail plates are characteristic. In some cases, erythroderma develops (which does not happen in CD), which usually regresses after 1–1.5 years. Histological examination of the affected skin reveals follicular hyperkeratosis. Horn plugs are located at the mouths of hair follicles and, unlike BC, do not penetrate deep into the epidermis and dermis. Perifollikulyarno visible areas of uneven moderate acanthosis, hypergranulosis. There is a slight perivascular lymphohistiocytic infiltrate in the upper dermis.

Like CD, keratoacanthoma multiplex (KA) is rare in clinical practice. Unlike CD, CA usually occurs in open areas of the skin (face, distal extremities). Lesions are small (1-3 mm in diameter) domed follicular papules with depression in the center, without a tendency to merge, in rare cases merging on the face. The color of the rash varies from flesh to pinkish red. Larger nodules have a characteristic appearance and evolution for CA. Dome-shaped papules have a depression in the center, filled with a horny plug. A characteristic feature of CA, in contrast to CD, is the spontaneous regression of lesions after 2–4 months with the formation of an atrophic scar. Histological examination reveals a peculiar architectonics characteristic of CA, resembling a “cup filled with horny masses”. In the epidermal outgrowths, atypia of cells, polymorphism, and the phenomenon of dyskeratosis are found. At the base of the lesion is a dense inflammatory cell infiltrate.

After differential diagnosis, based on clinical and morphological data, the patient was finally diagnosed with CD.

Isotretinoin therapy was started. From June 4, 2018 to January 29, 2019 (about 8 months), the patient received isotretinoin 30 mg/day (total received 7170 mg – 74.1 mg/kg). Against the background of the therapy, a pronounced positive trend was noted: the number of hyperkeratotic papules, comedo-like elements decreased, plaques flattened, their density and nodes significantly decreased, while the number of milia-like elements did not change significantly. In some areas in the thighs and knees, hair growth resumed. In the course of long-term treatment with isotretinoin, the patient developed side effects: retinoic dermatitis, blepharoconjunctivitis, dry eye syndrome, and rosacea symptoms intensified. The conclusion of the ophthalmologist (25.09.2018): severe dry eye syndrome during isotretinoin therapy. Blepharoconjunctivitis. Angiopathy of the retina. Background retinopathy and retinal vascular changes. To correct side effects, the patient receives additional therapy: dermatocosmetics (emollients), as well as therapy recommended by an ophthalmologist.

Taking into account the severity of side effects, since February 2019, the dose of isotretinoin was reduced to 10 mg / day, against which, after 2 months, new rashes typical of the disease began to appear. A follow-up consultation is currently planned for the patient to adjust the dosage of isotretinoin.

The observed by us middle-aged patient suffering from CD for a long time does not have serious concomitant diseases, which in some cases can be combined with this dermatosis (diabetes mellitus, kidney and liver diseases).