[Are multiple trichodiscomas/fibrofolliculomas the Birt-Hogg-Dubé syndrome?] / Mnozhestvennye trikhodiskomy/fibrofollikulomy ­ sindrom Berta­Khogg­D'yuba?

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.

[Experience with fractional photothermolysis in the therapy of gra-nuloma annulare]. / Opyt primeneniia fraktsionnogo fototermoliza v terapii kol'tsevidnoi granulemy.

BACKGROUND: Granuloma annulare is a benign inflammatory dermatosis of unknown etiology, which is characterized by the development of flesh-colored or red papules often arranged in rings on the skin. Currently there are no effective treatments for granuloma annulare. Topical and intradermal applications of glucocorticosteroids produce a short-term and insufficient therapeutic effect. The method cannot be used in extensive rashes. Fractional photothermolysis is a physical method for ablative exposure of tissues to an erbium laser via their local heating with light energy in certain areas. AIM: To investigate the efficiency of fractional photothermolysis in the therapy of patients with granuloma annulare. MATERIAL AND METHODS: Twenty-eight patients diagnosed with granuloma annulare were followed up. All the patients were treated with fractional Fotona Fidelis XS erbium laser at a wavelength of 2940 nm using a FS01 maniple. The treatment cycle consisted of 5 sessions. RESULT: After the treatment, the GASI index was decreased by 50, 75, and 100% in 5 (17.9%), 15 (53.6%), and 8 (28.6%) patients, respectively. The median GASI index was decreased by a total of 2.8 times (p<0.05). CONCLUSION: The study showed the high efficiency of fractional photothermolysis in the treatment of patients with granuloma annulare. This method can be recommended for the treatment of common forms of granuloma annulare.

[Anogenital mammary-like glands and related diseases. Part 2. Malignant tumors of the anogenital glands]. / Anogenital'nye mammaropodobnye zhelezy i sviazannye s nimi zabolevaniia. Chast' 2. Zlokachestvennye opukholi anogenital'nykh zhelez.

Mammary-like glands are a normal anatomical component of the anogenital region and can give rise to many benign and malignant tumors that morphologically mimic the similar diseases of the breast. The literature review is complemented by a description of 199 cases of malignant tumors of mammary-like glands. The paper presents the clinical and morphological characteristics of various malignant tumors of mammary-like glands, including extramammary Paget's disease, ductal, tubulolobular, adenoid cystic adenocarcinoma, low-grade phyllodes tumor, etc.

[Anogenital mammary-like glands and related lesions. Part 1. Benign tumors and tumor-like disorders]. / Anogenital'nye mammaropodobnye zhelezy i svyazannye s nimi zabolevaniya. Chast' 1. Dobrokachestvennye opukholi i opukholepodobnye protsessy anogenital'nykh zhelez.

Anogenital mammary-like glands represent a normal anatomic constituent of the anogenital area and may give rise to many benign and malignant tumors that morphologically mimic similar breast diseases. The literature review is complemented by a description of 286 cases of benign tumors and tumor-like processes in the mammary-like glands. The paper presents the clinical and morphological characteristics of papillary hidradenoma, fibroadenoma, benign phyllodes tumor, lactating adenoma, sclerosing adenosis, pseudoangiomatous stromal hyperplasia, etc.

Paediatric cutaneous lymphomas: a review and comparison with adult counterparts.

Primary cutaneous lymphomas (CL) in children is rare. Only a few studies focused specifically on paediatric CL and therefore little is known whether primary CL in children are similar to or different from their adult counterparts with respect to the clinicopathological presentation, behaviour and prognosis. An extensive literature search using PubMed/MEDLINE from January 1995 through July 2014 was undertaken for articles reporting cases of paediatric CL. In addition, we identified 31 children with CL in our institutions. Mycosis fungoides and lymphomatoid papulosis are the two most prevalent lymphoma forms in children. A few entities of cutaneous lymphomas such as cutaneous diffuse large B-cell lymphoma leg type, and Sézary syndrome have not been reported so far in children. Other lymphoma entities such as hydroa vacciniforme-like lymphoma are mostly seen in certain geographic areas (Asia, Central and South America). In the paediatric population, low-malignant indolent forms such as primary cutaneous marginal zone lymphoma and primary cutaneous follicle centre lymphoma are very rare, whereas the more aggressive forms of B-cell lymphomas, precursor lymphoblastic lymphomas, and blastic plasmacytoid dendritic cell neoplasm are the most common forms in children, mostly involving the skin secondarily. Most paediatric lymphomas have similar clinicopathological features and course as their adults counterparts, particularly in the group of cutaneous T-cell lymphomas. The spectrum of cutaneous B-cell lymphomas in children significantly differs from the one in adults. Diagnostic work-up and treatment of paediatric patients with lymphomas are best achieved in close collaboration with paediatric haematopathologists and oncologists.

[Diagnosis of cutaneous graft-versus-host disease: pathomorphological aspects].

OBJECTIVE: To study the differential diagnostic value of histological and immunohistochemical signs in skin biopsy specimens in graft-versus-host disease (GVHD). MATERIAL AND METHODS: Hematoxylin- and eosin-stained skin biopsy specimens from 50 patients with GVHD and 10 patients with toxicoderma were examined; immunohistochemical reactions using antibodies against CD3, CD20, CD4, CD8, FOXP3, CD56, CD1a, and CD68 were performed. The significance of differences between the groups was estimated using the chi2 and Mann-Whitney tests. RESULTS: In GVHD, there was a significantly high count of necrotic epidermocytes with satellite cells in the epidermis, more commonly apoptosis of epitheliocytes in the skin appendages and vacuolar dystrophy of epidermocytes. On the contrary, the count of eosinophilic granulocytes, CD1a+, CD3+, FOXP3+, and CD8+ cells and the proportion of FOXP3+ cells proved to be significantly higher in the toxicoderma group. CONCLUSION: The results of the investigation were used to form groups of signs that were important in the differential diagnosis of cutaneous GVHD and toxic and allergic reactions.

[Small-cell variant of CD30+ -anaplastic large-cell lymphoma of the skin].

Three cases of the so-called variant of primary cutaneous CD30+ anaplastic large cell lymphoma (ALCL) are presented. All patients were males aged 52, 59, and 78 years old; they had a solitary cutaneous tumor nodule. Their sites included the axilla, thigh, and shoulder. There was no extracutaneous involvement. Microscopically, the tumors were composed of small cells with irregular nuclei that were immunohistochemically positive for CD3, CD5, CD7, and CD30 and negative for B-cell markers; there was focal ALK-1 positivity in 1 case. Two cases had CD4+/CD8-phenotype, while the remaining one exhibited a CD4-/CD8+ immunoprofile. Fhedium to large CD30+ cells were rarely found scattered in the infiltrate. Monoclonal TCR gamma gene rearrangement was detected in 2 cases and rearrangement of IgH (lineage infidelity) was in one case. The tumors were surgically removed in all the patients. Two patients were alive and well 4 and 6 years after surgery, without evidence of cutaneous and extractaneous involvement (including the ALK+ patient). The third patient experienced several relapses of the skin tumor and developed axillary and inguinal lymph node involvement. Chemotherapy was performed and finally the patient underwent allogenic bone marrow transplantation; he died 3 years after the original diagnosis due to acute graft-versus-host disease and sepsis.

[Current approaches to diagnosing and treating primary cutaneous lymphomas on the basis of a new WHO-EORTC classification, cutaneous T-cell lymphomas].

This two-part review addresses the current diagnostic approaches towards primary cutaneous lymphomas. In this part, main T and NK-cell lymphomas are described with reference to clinical presentation, histological and immunohistochemical features, and genetic alterations based on the new WHO-EORTC classification of cutaneous lymphomas.

[Current approaches to the diagnosis and treatment of primary cutaneous lymphomas based on the new WHO-EORTC classification of B-cell cutaneous lymphomas].

The review concerns the current approaches to the diagnosis of primary cutaneous lymphomas. B-cell lymphomas are described with the reference to their clinical presentation, histological, and immunohistochemical features and genetic alterations based on the new WHO-EORTC classification of cutaneous lymphomas.

[Recurrent pigmented epidermotropic infiltrating breast ductular carcinoma simulating melanoma].

Recurrent pigmented breast cancer is a rare variant of this tumor and may clinically and microscopically simulate melanoma. A case of recurrent pigmented epidermotropic infiltrating breast ductular carcinoma is presented, by reviewing the literature on earlier published cases and discussing the pathogenesis of this condition.

Unusual manifestation of specific cutaneous involvement by B-cell chronic lymphocytic leukemia: spontaneous regression with scar formation.

We report a patient with specific cutaneous involvement by B-cell chronic lymphocytic leukemia, who demonstrated unusual clinical features during the course of the disease, namely several spontaneous regressions of skin lesions with the formation of scars. In addition, histologically proven keratoacanthoma was found. During the follow-up period of approximately 1.5 years, the patient experienced several recurrences of skin lesions and their partial spontaneous regression. The scars persisted and remained unchanged. We hypothesize that vascular injury combined with edema could have accounted for dermal ischemia and the subsequent development of the scarring lesions.

[The dynamic electrocardiogram in the late period after the surgical treatment of obesity patients]. / Dinamika élektrokardiogrammy v otdalennye sroki posle khirurgicheskogo lecheniia bol'nykh ozhireniem.

An analysis is presented of ECG of 68 patients with obesity (grade III-IV) before the operation of jejunoileal shunting and 1-2, 3-5 and 6-10 years after surgery. It was found that the main changes in the cardiovascular system occur during the first 2 years, i.e. during the period of body mass reduction. The importance of ECG in the diagnosis of hypertrophy of different compartments of the heart in obesity of grade III-IV.