Vol 26, No 3 (2023)

Otto–Moncorps keratosis is a rare dermatosis, which is excessive keratinization of the nipple and areola of the mammary gland with an unknown etiology and pathogenesis. According to the classification proposed by Levy–Frankel in 1938, the disease can be represented by three types: type 1 ― a variant of the epidermal nevus, type 2 ― associated with other diseases and type 3 ― idiopathic. The diagnosis is based on the data of the clinical picture, represented by diffuse keratinization of the nipple and areola of the mammary gland, dark brown in color, resembling basalt cubes. To date, there are no effective pathogenetic methods of treatment, methods of regular topical therapy are recommended (topical steroids and retinoids, keratolytics in high concentrations, emollients and moisturizers), as well as surgical methods of treatment (excision of hyperkeratotic overlays with a carbon dioxide laser, radiosurgical methods).

The article presents a unique case of hyperkeratosis of the periarticular circle and breast nipple type 3 according to Levy–Frankel in a 39-year-old patient. The diagnosis was made on the basis of anamnesis data (onset of the disease at puberty), a typical clinical picture (bilateral lesion; dermatosis in the form of hyperkeratotic layers resembling basalt cubes), as well as the characteristic course of the disease (relapse after discontinuation of therapy with local remedies). The interest of the presented clinical case lies not only in the rarity of this dermatosis and the duration of the disease history (for 24 years), but also in the presence of an extra-mammary lesion and a possible association with infertility of the patient.

BACKGROUND: Chronic urticaria is a skin disease characterized by the appearance of itchy weals and/or angioedema for 6 or more weeks. Chronic urticaria is subdivided into chronic spontaneous urticaria, which occurs due to an unknown cause, and chronic inducible urticaria, which occurs as a result of exposure to various physical factors (water, cold, heat, pressure, mechanical irritation), can occur simultaneously or independently of each other. Omalizumab, anti-IgE monoclonal antibody, is approved for the treatment of patients with chronic spontaneous urticaria and is the second choice in cases of resistance to antihistamine treatment. In patients with a combination of chronic spontaneous urticaria and chronic inducible urticaria, the effectiveness of treatment with omalizumab has been little studied.

AIM: compare the effectiveness of omalizumab treatment in patients with chronic spontaneous urticaria and patients with a combination of chronic spontaneous and chronic induced urticaria.

MATERIALS AND METHODS: Under supervision there were 30 patients with chronic spontaneous urticaria and combined chronic spontaneous and inducible urticaria (15 patients in each group). Evaluation of the effectiveness of treatment was carried out according to the results of the questionnaires DLQI (dermatological index of quality of life), CU-Q2oL (questionnaire for quality of life in chronic urticaria), UCT (urticaria control test), UAS (urticaria activity scale), HADS (Hospital Anxiety and Depression Scale) and provocation tests in dynamics before and during treatment.

RESULTS: All patients received omalizumab 300 mg subcutaneously once a month for 6 to 12 months. After the first injection of omalizumab, we noted a decrease in the severity of urticaria, an increase in the level of disease control and quality of life when comparing parameters before and during treatment in more than 90% of patients. Improved performance remained at this level throughout all subsequent months of treatment.

CONCLUSION: Omalizumab is equally effective in patients with an isolated form of chronic spontaneous urticaria and in patients with a combined form of chronic spontaneous and inducible urticaria. The use of omalizumab allows you to control the symptoms of chronic spontaneous and inducible urticaria, even with prolonged use.

Vitiligo is the most common acquired skin depigmentation disorder characterized by progressive loss of pigmentation caused by the destruction of functional melanocytes in the epidermis. The pathogenesis consists in the interaction of genetic components, metabolic factors associated with cellular oxidative stress, adhesion of melanocytes to the epithelium and autoimmunity, which culminate in aggression against melanocytes. To date, the treatment of vitiligo, according to Russian and European clinical guidelines, consists in the appointment of various drug and non-drug methods: the use of topical and systemic glucocorticosteroids, calcineurin inhibitors, azathioprine, phototherapy, both in the form of monotherapy and their combination.

In recent years, in connection with the development of cellular technologies, alternative methods of therapy based on the transplantation of autologous cultured and non-cultured melanocytes have become widespread in the treatment of vitiligo. The most promising options for vitiligo cell therapy are methods based not only on the transplantation of ready-made cellular structures, but also on the replacement of damaged cells with a transplant of pluropotent progenitor stem cells or their immature committed structures.

This article is of an overview nature. The aim of the review is to update information on promising new treatments for vitiligo. A literature review was conducted using the PubMed, Cochrane Library, CyberLeninka and Internet databases to study clinical and preclinical data on the possibility of using innovative methods of regenerative medicine in patients with vitiligo.

This review is addressed to medical researchers interested in the treatment of vitiligo.

Acne treatment has been revolutionized since 1982 when isotretinoin was approved for the first time for the condition.

Isotretinoin (13-cis-retinoic acid) is still the most effective treatment for acne. Systemic retinoid (a derivative of vitamin A) is the only drug acting on all links of the pathogenesis of this disease. Isotretinoin is believed to improve the condition of the skin in acne by reducing sebum production by the sebaceous glands, normalizing follicular keratinization and suppressing the growth of Actinobacterium acnes (Propionibacteriaceae family) by changing the follicular environment and reducing inflammation.

Despite being widely prescribed by doctors for almost forty years, there is still a lot of uncertainty about how best to prescribe this drug, how long the remission lasts, and what side effects there may be. Isotretinoin has been proven to be highly effective and well tolerated, but like any medicine, it also has certain side effects, but it should be understood that they dose-dependent and reversible.

We provide up-to-date information on the possible side effects of isotretinoin and discuss ways to prevent and treat these side effects.

BACKGROUND: Atopic dermatitis is characterized by a clear upward trend in the prevalence and development of severe forms of dermatosis, which represents an important medical and social problem.

AIM: Pathogenetic justification and study of clinical efficacy of combined use of dupilumab and cyclosporine in children with severe refractory atopic dermatitis.

MATERIALS AND METHODS: The study included 30 children and adolescents over 6 years of age, with a diagnosis of atopic dermatitis with severe disease severity. The control group consisted of 15 people. EASI, Pruritus VAS score, CDLQI index were used to assess the dynamics of disease symptoms. The content of interleukins in blood serum was determined by enzyme immunoassay. Children included in the study were divided into two groups of 15 patients by randomization. Group 1 patients received dupilumab monotherapy, Group 2 patients were treated with a combination of dupilumab and cyclosporine.

RESULTS: Positive dynamics in the course of the cutaneous pathological process was observed in all patients. A more pronounced and persistent tendency to further decrease of EASI values was preserved in patients receiving combined therapy. During dupilumab monotherapy and combined treatment there was a significant reduction in nasal obstruction, a decrease in the number of bronchial obstruction/cough attacks. In the course of therapy in all patients it was possible to achieve a significant reduction in the negative impact of atopic dermatitis on the quality of life of children and adolescents. Dupilumab therapy contributed to normalization of cytokine status, eosinophil levels and total immunoglobulin E. However, after 12 weeks of dupilumab monotherapy, signs of Th1-response activation were revealed.

CONCLUSION: Combined therapy reduces the duration of treatment with dupilumab to 12 weeks, avoids side effects and complications, and significantly increases clinical efficacy of therapeutic measures in children and adolescents with severe atopic dermatitis.

On May 12, 2023, 40-th Rakhmanov readings were held in Moscow. The conference, which has long become traditional, has now become a major interdisciplinary and international forum bringing together representatives of not only therapeutic, but also aesthetic medical specialties.

The program of the anniversary conference "Rakhmanov readings 40 years" was rich: it covered new trends in dermatology and cosmetology; issues of diagnosis, treatment, prevention and rehabilitation of dermatoses. At the same time, the participants got acquainted and established interdisciplinary contacts.

In total, 11 sections, 10 symposiums, 1 quiz were held within the framework of the scientific and practical conference "Rakhmanov Readings"; 100 scientific reports on dermatovenerology and cosmetology were presented.

In continuation of orphan dermatosis, I would also like to say about diseases such as lichen planus ― which is an autoimmune disease in which the expression of an antigen that has not yet been identified by keratinocytes of the basal layer leads to the activation and migration of T-lymphocytes into the skin with the formation of an immune response and an inflammatory reaction.

Bourneville–Pringle syndrome (tuberous sclerosis complex) is a rare genetic disease that is part of the group of phakomatoses, characterized by a polysystemic lesion involving the skin, central nervous system, organs of vision, etc. in the pathological process.

Bullous scleroderma is a rare type of localized scleroderma with a predominant skin lesion in the form of foci of induration and sclerosis, subepidermal blisters with serous contents.

Trichoadenoma is a rare skin tumor belonging to the group of benign follicular tumors. Consists of numerous, relatively large, round or oval funnel-shaped structures separated by an underdeveloped fibrous stroma.

Pseudolymphoma is a reactive polyclonal T- or B-cell lymphoproliferative process that can manifest as both localized and disseminated skin lesions.

Giant flaming nevus of the skin of the face is usually a benign disease, but in some cases malignancy is possible.

T-cell anaplastic lymphoma associated with sarcoidosis is characterized by lymphadenopathy, lesions, circulating abnormal lymphocytes, and hypercalcemia leading to the sarcoidosis-lymphoma syndrome.

Devergy's disease is caused by a violation of keratinization and the appearance of follicular hyperkeratotic papules, palmoplantar hyperkeratosis and erythematous scaly plaques with an orange tint.

Favre–Shay ocher yellow dermatitis occurs in patients with varicose ulcers on the inner surface of the lower third of the legs, which it either precedes or accompanies.

Sweet's febrile neutrophilic dermatosis manifests itself in three clinical cases: classical (the disease is preceded by an infectious disease), malignant (occurs as a paraneoplastic syndrome), medicinal (most often occurs in patients treated with G-CSF).

Lipodermatosclerosis is manifested by induration and hyperpigmentation of the skin, affecting one or both legs, in a characteristic "upside down champagne bottle" appearance.