Granulomatous cheilitis is a chronic swelling of one or both lips that is caused by granulomatous inflammation. This is a rare inflammatory disease that belongs to the monosymptomatic form of Melkersson–Rosenthal syndrome. To date, the etiology of this disease remains unknown, and its treatment is challenging. The article discusses a case report of a monosymptomatic variant of recurrent orofacial edema, describes the features of the observed case and efficacy of intradermal application of glucocorticosteroids and fractional photothermolysis, and provides data on the differential diagnosis of the disease. This case highlights the importance of thorough examination in the diagnosis process as the clinical presentation can be similar to many other granulomatous conditions.

ABSTRACT:Nonmelanoma malignancies associated with congenital melanocytic nevi (CMN) are extremely rare, with only 12 reported cases of rhabdomyosarcoma (RMS) to date. We present 2 additional cases of RMS arising in giant CMN, with immunohistochemical and molecular biologic investigations. The first case was a 32-year-old woman with a personal history of melanoma in giant CMN who, after successful treatment and long remission, presented with a new 1-cm nodule within the CMN. Microscopically, the atypical areas exhibited a round cell/alveolar morphology with immunoreactivity for desmin and myogenin, and lacked PAX3/7::FOXO1 fusions typical for alveolar RMS on a reverse transcription polymerase chain reaction analysis. An identical NRAS p.Q61R mutation with comparable variant allele frequency (32% and 44%) was identified in both the nevus and the RMS tissue by next-generation sequencing. The second patient was a 5-year-old girl with a rapidly growing, bleeding, ulcerated 3 × 4 cm interscapular mass within a giant CMN that histologically seemed as a proliferation of pleomorphic spindle, polygonal and epithelioid cells with marked pleomorphism immunoreactive for myogenin, muscle-specific actin, and smooth muscle actin. Next-generation sequencing yielded an HRAS p.Q61R mutation with limited variant allele frequency (7%) in the RMS component, while ATRX p.Q2193* variant was detected in the nevus. Our study is apparently the first report of NRAS and HRAS mutations in tumors with RMS phenotype arisen in CMN.

BACKGROUND:Pediatric Mycosis fungoides (MF) management extrapolates from adult guidelines, despite differing clinical aspects. Recommendations are essential to address unique challenges in this distinct patient group. OBJECTIVE:This project aims to derive consensus recommendations for pediatric MF management. METHODS:Experts from pediatric dermatology, general dermatology, dermatopathology, and pediatric hematology-oncology (N = 83) were invited to contribute to consensus recommendations. The process involved 3 electronic Delphi rounds, concluding with a final consensus meeting using a modified Nominal Group Technique for unresolved items. RESULTS:Consensus included more clinical severity measures than tumor-node-metastasis-blood staging: pruritus, functional or esthetic impairment (eg, palms, soles, genitalia), quality of life impact, and psychological aspects (eg, embarrassment, anxiety, depression), plus parental anxiety. Ten recommendations were made for managing early and advanced pediatric MF. Disagreement emerged in choosing therapies beyond stage I of the disease. DISCUSSION:This multinational initiative aimed to standardize optimal pediatric MF management and successfully generated consensus recommendations. Additional work is needed for structured, prospective protocols in advanced-stage pediatric MF. LIMITATIONS:Lack of pediatric hematologists-oncologists and patients' representatives. CONCLUSION:Documentation of extended clinical severity and outcome measures is recommended. Addressing the need for structured protocols in advanced-stage pediatric MF and implementing systematic, prospective data collection is crucial.

Close relationship between melanocytes and neural cells is accepted to reflect their common derivation from the neural crest and tumors combining both elements. We present a series of 10 patients with giant congenital melanocytic nevi (CMN) in which a secondary proliferation (11 lesions) with schwannian and/or perineuriomatous differentiation developed in the course of the disease. The age of the patients (4 male and 6 female) at the time of surgery and histological assessment varied from 3 months to 57 years. Histopathologically, the following subgroups were delineated: (1) nodular/tumoriform "neurotization" in CMN, (2) diffuse neurofibroma-like proliferation within CMN, (3) plexiform neurofibroma-like proliferation within CMN, and (4) diffuse perineuriomatous (hybrid schwannomatous-perineuriomatous) differentiation in CMN. We review the pertinent literature, including the role of recently identified Schwann cell precursors which are believed to represent the nerve-associated state of neural crest-like cells that persists into later developmental stages.

A clinical case of lymphomatoid papulosis in a 10-year-old boy is described, clinically characterized by the presence of ulcerating nodes that resolve with the atrophy formation. The patient’s differential diagnostic workup included cutaneous anaplastic large cell lymphoma, cutaneous tuberculosis, polyarteritis nodosa, erythema nodosum, deep lupus erythematosus, and alpha-1 antitrypsin deficiency. Based on the history, clinical picture, as well as laboratory and instrumental studies, the patient was diagnosed with lymphomatoid papulosis. During the examination, no concomitant neoplastic diseases were identified in the patient. All elements resolved with the outcome being hyperpigmented spots and atrophy. To date, the relapse-free period of the disease in the patient is 10 months.

A case of idiopathic eruptive macular pigmentation in a child demonstrates a variant of skin hyperpigmentation of uncertain etiology. We described an 11-year-old boy with light brown spots in the torso and limbs that developed against the background of normal skin without an established provoking factor. On examination, it was found that the disease had developed within one year. Initially, the process was localized to the elbow folds and axillary areas, but later the rash spread to the trunk, neck and extremities. No inflammatory changes were observed at the base of the rash elements. The patient was examined for the presence of diseases that could underlie the skin manifestations, and histological examination was performed to verify the diagnosis. The established diagnosis of idiopathic eruptive macular pigmentation made it possible to choose a wait-and-see approach in treating the patient, due to the available literature data on the self-resolution of this pathology and ineffectiveness of the available dermatosis treatment options.

Background. Primary cutaneous lymphomas are the second most common group of extranodal lymphomas. Unlike nodal lymphomas, where B-cell proliferations dominate, primary cutaneous T-cell lymphomas account for 6575% of all cutaneous lymphomas. Among T-cell lymphomas of the skin, about 50% of cases are mycosis fungoides (MF), the second place in frequency of occurrence is occupied by CD30-positive lymphoproliferative skin diseases (CD30 LPD), about 10% are rare nosological forms, such as primary cutaneous peripheral T-cell lymphoma, unspecified, Sezari syndrome (SS), etc. During the initiating treatment of patients with MF and Szary syndrome (SS), carried out on the territory of the Russian Federation, for about 30% of patients are resistant to various therapeutic effects, especially in the later stages. The problem of the treatment of CD30+ LPD is extracutaneous dissemination in case of primary cutaneous anaplastic large cell lymphoma (pcALCL), steadily relapsing course of lymphomatoid papulosis (LyP) without symptom-free intervals. These characteristics of the therapy of cutaneous lymphomas demand for the need to search for new treatment options. Brentuximab vedotin, according to the results of the international randomized ALCANZA trial, has shown high efficiency in the treatment of cutaneous T-cell lymphoproliferative diseases. Aim. To evaluate the efficacy of brentuximab vedotin application in patients with cutaneous T-cell lymphomas in adverse risk group received at least one line of systemic therapy. Materials and methods. The study included 21 patients: 16 men and 5 women. The diagnosis of MF was verified in 8 patients, SS in 5 patients, cutaneous CD30+ LPD in 6 patients (5 patients pcALCL, 1 patient LyP) and a primary cutaneous peripheral T-cell lymphoma, unspecified in 2 patients. The diagnosis of cutaneous T-cell lymphoma was verified on the basis of the anamnesis of the disease, on the character of cutaneous lesions, on histological, immunohistochemical and in some cases on molecular genetic testing of the skin biopsy (the assessment of T-cell receptor gene rearrangement). Results. The late stages of the disease were diagnosed in 12 of 13 patients with MF/SS. Extracutaneous lesions were diagnosed in 57% of cases. The median of prior lines therapy was 3 (18 variants of treatment). The overall response to the treatment was achieved in 91% of cases (in 19 of 21 patients): the complete remission was obtained in 53% of cases, very good partial remission in 31% of cases and partial remission in 16% of cases. The progression of the disease was determined in 2 patients (after the first and fourth cycles). Some patients with partial remission as a result of therapy using brentuximab vedotin had the additional therapy (radiation therapy, interferon , the cycles of systemic therapy) and these acts gave an option of achieving deeper antitumor response. The early relapse was diagnosed in 2 of 19 patients who had responded to the treatment. The treatment tolerability was acceptable, and the toxicity did not exceed the already known one described in earlier studies. Thus, the stable overall antitumor response had been persisting in 89% of patients (the median of the observation was 10 months). Conclusion. The use of targeted therapy with brentuximab vedotin gave an option of achieving high treatment results in group of patients with advanced stages of the disease and inefficiency of several lines of therapy.

The article is devoted to a rare disease of cutaneous collagenous vasculopathy (CCV), which is characterized by common leather telangiectasias and specific histological signs: extended capillaries in the surface layers of the dermis, the walls of which are thickened due to hyaline deposits containing type IV collagen. According to literature, there are no publications about CCV in Russian sources. In foreign literature, only 60 cases are described, the first of which is mentioned in 2000. The patient turned to the EXCLUSIVE medical clinic to resolve the issue of the tactics of treatment of chronic HCV-infection 1b subtype and complaints of common telangiectasia on the skin and mucous membrane of both sclera, without subjective sensations. The first rashes appeared in 2008 and gradually progressed. When performing histological examination, expanded capillaries were found in the surface layers of the dermis, the walls of which are thickened due to protein deposits containing type IV collagen. The described case of CCV is the first in Russian literature and shows the need for histological and immunohistologycal studies to establish a final diagnosis.

In connection with the upcoming transition to the International Classification of Diseases and Health-related Problems of the eleventh revision (ICD-11), the authors of the article propose to replace the term toxidermy with a new, widely used in the world term drug-induced skin reactions. The article presents a list of various variants of drug-induced rashes, which are included in the draft ICD-11. For standardization of definitions and diagnostic criteria, a unified working classification of this group of diseases is based on a mixed principle clinical manifestations (primary morphological elements of skin rash), etiological and pathogenetic aspects are taken into account. The applied unified classification of drug-induced skin reactions is proposed for discussion. Examples of the formulation of clinical diagnoses are given. Severe drug-induced skin reactions are described, which are potentially life-threatening for the patient, can lead to disability and require mandatory hospitalization.

The article is devoted to a rare variant of skin sarcoidosis the so-called follicular sarcoidosis. According to the literature, the follicular form of sarcoidosis is extremely rare, several isolated cases are described in foreign literature, there are no publications in the russian literature. A 30-year-old female patient came to the clinic of the Military Medical Academy with a skin rash of the upper extremities, trunk and neck that lasted for one year. Anamnesis: repeated use of topical corticosteroids for the treatment with a temporary positive effect. The skin process was represented by common follicular papules; a histological examination of one of the elements revealed granulomas of the sarcoid type, which were located perifolliculary, mainly around the infundibular sections of the hair follicles. The rash resolved after a course of external corticosteroid therapy. The clinical case described by us illustrates the need for histological examination to verify the diagnosis.

Background. Mastocytosis is a disease caused by proliferation and accumulation of clonal mast cells in one or more organs. It is often associated with other hematological tumors. Aggressive forms of mastocytosis (AFM) require specific therapy. In non-aggressive forms of mastocytosis (NFM) symptomatic treatment is needed. Aim. To analyze the clinical course and treatment outcomes in different forms of adult mastocytosis. Materials & Methods. The retrospective analysis was based on the records of patients who received in-person and distance consultation within the period from 11/2008 to 11/2020. The analysis of complaints in disease onset and over time was carried out using questionnaires. NFM patients received symptomatic treatment with antihistamines. To all AFM patients chemotherapy was administered. Results. The analysis includes the data of 58 patients: 39 (67.2 %) women and 18 (32.8 %) men. The median age was 40 years (range 18-79 years), the median age on diagnosis was 39 years (range 1-79 years). In all patients skin rashes were reported. The median age of the first skin manifestations was 25 years (range 0.1-70 years). In-person monitoring was conducted in 34 (58.6 %) patients, 24 (41.4 %) patients received distance consultations. Median follow-up was 56.5 months (range 3-564 months). In 8 (13.7 %) patients mastocytosis was diagnosed in childhood with the median of 9 years (range 0-15 years). The diagnosis was morphologically confirmed in 46 (79.3 %) patients. Main complaints included pruritus (67.2 %), edema and erythema response to various irritants (62 %). In 45 (77.5 %) patients NFMs were reported. The regular symptomatic treatment of 78.8 % of NFM patients consisted only of antihistamines (57.9 %), and 2 (4.4 %) patients noted poor disease symptom control. One (2.2 %) patient died of associated chronic myelomonocytic leukemia. None of NFM patients required cytoreductive treatment. AFMs were diagnosed in 13 (22.4 %) patients, 5 (38.4 %) out of them had mast cell leukemia. The indications for starting chemotherapy were cytopenia (n = 3; 23 %), extensive osteolysis (n = 7; 53.8 %), ascitic syndrome with portal hypertension (n = 6; 46.1 %). Overall survival of AFM patients was 84.6 % (n = 11) with median follow-up of 80 months (range 12-131 months). Conclusion. NFM prognosis is favorable. Antihistamines are effective in relieving complaints of most patients. Cytostatic treatment of AFM in some patients provides long-lasting antitumor response.

Analysis of various classifications of pemphigus shows that there are no fundamental differences between them. The main distinctions consist in use of diverse terms in naming of some forms of pemphigus and in inclusion or exclusion of certain subtypes from the classifications. Authors propose to use the following classification in the dermatological clinical practice, for educational and scientific purposes and for clinical guidelines: 1) pemphigus vulgaris (1.1. Pemphigu s vegetans); 2) pemphigus foliaceus (2.1. Pemphigus endemic (Fogo selvagem), 2.2. Pemphigus erythematosus (Senear Usher)); 3) herpetiform pemphigus; 4) paraneoplastic pemphigus; 5) IgA pemphigus (5.1. Subcorneal pustular dermatosis, 5.2. Intraepidermal neutrophilic dermatosis).

Background. Primary cutaneous T-cell lymphomas are rare heterogeneous group of lymphoproliferative diseases characterized by primarily involving skin and subcutaneous adipose tissue. Half of these cases are mycosis fungoides (MF), for about 25% are cutaneous CD30+ lymphoproliferative diseases (CD30+ LPD): primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis (LyP). During the initiating treatment of patients with MF and Szary syndrome (SS), carried out on the territory of the Russian Federation, for about 30% of patients are resistant to various therapeutic effects, especially in the later stages. The problem of the treatment of CD30+ LPD is extracutaneous dissemination in case of pcALCL, steadily relapsing course of LyP without symptom-free intervals. These characteristics of the therapy of cutaneous lymphomas demand for the need to search for new treatment options. Brentuximab vedotin, according to the results of the international randomized ALCANZA trial, has shown high efficiency in the treatment of cutaneous T-cell lymphoproliferative diseases. Aim. To evaluate the efficacy of brentuximab vedotin application in patients with cutaneous T-cell lymphomas in adverse risk group received at least one line of systemic therapy. Materials and methods. The study included 21 patients: 16 men and 5 women. The diagnosis of MF was verified in 8 patients, SS in 5 patients, cutaneous CD30+ LPD in 6 patients (5 patients pcALCL, 1 patient LyP) and a primary cutaneous peripheral T-cell lymphoma, unspecified in 2 patients. The diagnosis of cutaneous T-cell lymphoma was verified on the basis of the anamnesis of the disease, on the character of cutaneous lesions, on histological, immunohistochemical and in some cases on molecular genetic testing of the biopted sample of the skin (the assessment of T-cell receptor gene rearrangement). Results. The late stages of the disease were diagnosed in 12 of 13 patients with MF/SS. Extracutaneous lesions were diagnosed in 57% of cases. The median of prior lines therapy was 3 (18 variants of treatment). The overall response to the treatment was achieved in 91% of cases (in 19 of 21 patients): the complete remission was obtained in 53% of cases, very good partial remission in 31% of cases and partial remission in 16% of cases. The progression of the disease was determined in 2 patients (after the first and fourth cycles). Some patients with partial remission as a result of therapy using brentuximab vedotin had the additional therapy (radiation therapy, interferon , the cycles of systemic therapy) and these acts gave an option of achieving deeper antitumor response. The early relapse was diagnosed in 2 of 19 patients who had responded to the treatment. The treatment tolerability was acceptable, and the toxicity did not exceed the already known one described in earlier studies. Thus, the stable overall antitumor response had been persisting in 89% of patients (the median of the observation was 10 months). Conclusion. The use of targeted therapy with brentuximab vedotin gave an option of achieving high treatment results in group of patients with advanced stages of the disease and inefficiency of several lines of therapy.

Birt-Hogg-Dubé syndrome is a rare autosomal dominant disease caused by a mutation in the FLCN gene and presents with a triad of multiple fibrofolliculomas, trichodiscomas, and masses that clinically resemble fibroepithelial polyps (acrochordones), accompanied by an increased risk of kidney tumors and lung cysts. The paper provides a literature review supplemented by clinical cases and the morphological pattern of skin lesions. It presents the clinical and morphological features of cutaneous manifestations of the syndrome and gives diagnostic criteria.

RosaiDorfman disease (RDD) is a rare variant of the nonlangergans histiocytosis. Various presentation, systemic and localised forms and limited publications make diagnostics and prompt management difficult. Aim. Literature review and presentation of the patient with cutaneous form of RDD. Results. The patient is a 56 y.o male. In October 2019 he noticed a tumour in the left temporal area. After 3 weeks the tumor was removed. During the next two weeks the tumour recurred within the post-operative scar. After the review of the specimen and staging the skin form RosaiDorfman disease was diagnosed. Irradiation (total dose 36 Gr) was conducted. The tumor lessened. Through the next 4 months response is stable. Сonclusion. Radiation therapy as a second line of treatment of the skin RDD led to a stable response.

Актуальность. Мастоцитоз — заболевание, обусловленное пролиферацией и накоплением клональных тучных клеток в одном или нескольких органах. Часто заболевание протекает в ассоциации с другими опухолями системы крови. Пациенты с агрессивными формами мастоцитоза (АФМ) нуждаются в специфической терапии. Пациентам с неагрессивными формами мастоцитоза (НФМ) необходимо симптоматическое лечение. НФМ преобладают, поэтому заболевание часто остается нераспознанным. Цель. Анализ клинического течения и результатов терапии у взрослых пациентов с различными формами мастоцитоза. Материалы и методы. Для ретроспективного анализа использовались медицинские документы пациентов, обратившихся очно и консультированных дистанционно в период 11.2008–11.2020 гг. Анализ жалоб в дебюте заболевания и в динамике проводился с помощью анкетирования. Больные с НФМ получали симптоматическую терапию антигистаминными средствами. Все пациенты с АФМ получали противоопухолевое лечение. Результаты. Проанализированы данные 58 пациентов: 39 (67,2 %) женщин и 18 (32,8 %) мужчин. Медиана возраста составила 40 лет (диапазон 18–79 лет), медиана возраста при постановке диагноза — 39 лет (диапазон 1–79 лет). У всех пациентов отмечались кожные высыпания. Медиана возраста на момент появления кожных проявлений составила 25 лет (диапазон 0,1–70 лет). Под непосредственным наблюдением находилось 34 (58,6 %) пациента, 24 (41,4 %) — консультированы дистанционно. Медиана наблюдения составила 56,5 мес. (диапазон 3–564 мес.). У 8 (13,7 %) пациентов мастоцитоз был диагностирован в детском возрасте, медиана 9 лет (диапазон 0–15 лет). Морфологически диагноз был подтвержден у 46 (79,3 %) пациентов. Основными жалобами были кожный зуд (67,2 %), отек и покраснение высыпных элементов в ответ на различные раздражители (62 %). У 45 (77,5 %) больных наблюдались НФМ. В качестве регулярной симптоматической терапии 78,8 % пациентов с НФМ получали только антигистаминные средства (57,9 %), при этом 2 (4,4 %) пациента отметили неудовлетворительный контроль над симптомами болезни. 1 (2,2 %) пациент скончался от ассоциированного хронического миеломоноцитарного лейкоза. Течение мастоцитоза у всех больных с НФМ не потребовало проведения циторедуктивного лечения. АФМ диагностированы у 13 (22,4 %) пациентов, из них у 5 (38,4 %) имел место тучноклеточный лейкоз. Показанием к началу противоопухолевой терапии были цитопении (n = 3; 23 %), активный остеолитический процесс (n = 7; 53,8 %), отечно-асцитический синдром с портальной гипертензией (n = 6; 46,1 %). Общая выживаемость в группе с АФМ составила 84,6 % (n = 11) при медиане наблюдения 80 мес. (диапазон 12–131 мес.). Заключение. Прогноз у пациентов с НФМ благоприятный. Антигистаминные средства эффективны для купирования жалоб у большинства больных. Цитостатическое лечение при АФМ позволяет достичь длительного противоопухолевого ответа у части больных.

Primary cutaneous T-cell lymphomas are the heterogeneous group of T-cell lymphoproliferative diseases (LPD), which are developed mainly in the skin and characterized by diagnostic features, clinical course and therapeutic approach. Primary cutaneous anaplastic lymphoma (с-ALCL) and lymphomatoid papulosis (LyP) are diagnosed In a quarter of cases of all T-cell skin lymphomas. The tumor cells in primary cutaneous CD30-positive skin lymphomas express CD30 in more than 75% of cases. The majority of patients with cutaneous CD30+ LPD have an indolent course with a favorable prognosis, the resistant course of disease develops in about 30% of cases and fatal cases from lymphoma are registered in 8% of cases. Most commonly, the treatment of these forms of LPD includes the surgical removal, radiation therapy or small doses of methotrexate, the systemic chemotherapy is used to generalize the process. Recently, monoclonal antibodies have been included in clinical practice for the treatment of skin lymphomas, one of which is brentuximab vedotin, the use of which has shown a rather high efficiency in the treatment of CD30 + skin lymphomas: more than 2/3 of the patients responded to the treatment, despite the many lines of therapy (median of prior effect is 3.1 for c-ALCL). The general group of patients with cutaneous T-cell lymphomas and who received the consultative-diagnostic or inpatient treatment at the National Research Centre of Hematology includes 328 patients. Among them the CD30-positive cutaneous LPD were verified in 33 patients (10%): 16 patients with LyP, 17 patients with c-ALCL. The eruptions were regressed on its own without specific therapy in 75% of patients with LyP and 4 patients received the treatment. As well as one patient with the relapsing course of disease, with a long period of self-regression of the papules, with the lack of the treatment effect (phototherapy and low-dose methotrexate) received brentuximab vedotin (BV). The complete clinical response was achieved after the first cycle of monotherapy with BV and persists for more than a year. Patients with c-ALCL more frequently needed the specific therapy (76% of patients), only 4 patients were observed with the self-regression of the papules within 6-9 weeks. Most patients received the interferon alfa therapy with the complete clinical response; the rest patients - local radiation therapy, the small doses of methotrexate. The systemic chemotherapy was done to the 5 patients. Considering the common process, however 2 of them died from infectious complications during the chemotherapy, 2 patients had early recurrence and only 1 patient is in the long-lasting complete remission after the high-dose of chemotherapy. In summary, the primary cutaneous CD30 + lymphoproliferative diseases cover a spectrum of benign condition with the so-called "malignant" phenotype. LyP and c-ALCL have a favorable prognosis, 5-year survival rate is exceeding 95%, photo- and immunotherapy are successfully used in the treatment, while the use of systemic chemotherapy should be avoided (shortening of the remissions duration, progression of the infectious complications). The use of targeted therapy (brentuximab vedotin) increases the therapeutic ability in the situations where the routine treatment options are ineffective. Primary cutaneous T-cell lymphomas are the heterogeneous group of T-cell lymphoproliferative diseases (LPD), which are developed mainly in the skin and characterized by diagnostic features, clinical course and therapeutic approach. Primary cutaneous anaplastic lymphoma (с-ALCL) and lymphomatoid papulosis (LyP) are diagnosed In a quarter of cases of all T-cell skin lymphomas. The tumor cells in primary cutaneous CD30-positive skin lymphomas express CD30 in more than 75% of cases. The majority of patients with cutaneous CD30+ LPD have an indolent course with a favorable prognosis, the resistant course of disease develops in about 30% of cases and fatal cases from lymphoma are registered in 8% of cases. Most commonly, the treatment of these forms of LPD includes the surgical removal, radiation therapy or small doses of methotrexate, the systemic chemotherapy is used to generalize the process. Recently, monoclonal antibodies have been included in clinical practice for the treatment of skin lymphomas, one of which is brentuximab vedotin, the use of which has shown a rather high efficiency in the treatment of CD30 + skin lymphomas: more than 2/3 of the patients responded to the treatment, despite the many lines of therapy (median of prior effect is 3.1 for c-ALCL). The general group of patients with cutaneous T-cell lymphomas and who received the consultative-diagnostic or inpatient treatment at the National Research Centre of Hematology includes 328 patients. Among them the CD30-positive cutaneous LPD were verified in 33 patients (10%): 16 patients with LyP, 17 patients with c-ALCL. The eruptions were regressed on its own without specific therapy in 75% of patients with LyP and 4 patients received the treatment. As well as one patient with the relapsing course of disease, with a long period of self-regression of the papules, with the lack of the treatment effect (phototherapy and low-dose methotrexate) received brentuximab vedotin (BV). The complete clinical response was achieved after the first cycle of monotherapy with BV and persists for more than a year. Patients with c-ALCL more frequently needed the specific therapy (76% of patients), only 4 patients were observed with the self-regression of the papules within 6-9 weeks. Most patients received the interferon alfa therapy with the complete clinical response; the rest patients - local radiation therapy, the small doses of methotrexate. The systemic chemotherapy was done to the 5 patients. Considering the common process, however 2 of them died from infectious complications during the chemotherapy, 2 patients had early recurrence and only 1 patient is in the long-lasting complete remission after the high-dose of chemotherapy. In summary, the primary cutaneous CD30 + lymphoproliferative diseases cover a spectrum of benign condition with the so-called "malignant" phenotype. LyP and c-ALCL have a favorable prognosis, 5-year survival rate is exceeding 95%, photo- and immunotherapy are successfully used in the treatment, while the use of systemic chemotherapy should be avoided (shortening of the remissions duration, progression of the infectious complications). The use of targeted therapy (brentuximab vedotin) increases the therapeutic ability in the situations where the routine treatment options are ineffective.