Vol 17, No 3 (2014)


Molecular mechanisms of development of basal-cell carcinoma associated with hereditary syndromes

Snarskaya E.S., Poluboyarov A.A.


New data on the pathogenetic mechanisms leading to the development of epithelial neoplasm of the skin, such as Hedgehog and Wnt signal pathways, were obtained recently. Impairments of the molecular mechanisms in these signal pathways were detected for the first time in some hereditary diseases and various tumors, including cutaneous basal-cell carcinoma. It was for the first time revealed that Hedgehog and Wnt signaling regulated the activities of the main genes involved in the morphogenesis processes. Gorlin-Goltz genetic syndrome is associated with early development of basal-cell cutaneous cancer. The genetic base and the molecular mechanisms of the pathogenesis, causing the development of multiple tumors in this syndrome, are discussed. The genetic factors promoting a higher incidence of chromosome aberrations simultaneously promote the development of cancer.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):4-8
pages 4-8 views

Clinical observation of Muir-Tor-rais's syndrome

Molochkov V.A., Kuntsevich Z.S., Dibirova S.D., Molochkova Y.V., Kazantseva K.V.


Muir-Torrais ’ syndrome in a 60-year-old patient with a history of rectal adenocarcinoma and papillary cancer of the renal pelvis is described. The syndrome included corneal keratoacanthoma, sebaceous gland adenoma, and rectal adenocarcinoma. Keratoacanthoma was cured by peritumor injections of reaferon (interferon-a2b, 1.5 • 10 6 U every other day, 9 injections per course). Rectal cancer, diagnosed at the early stage, was effectively treated by surgery. During the subsequent 2 years the patient developed gastric cancer, which was also treated surgically with good results. The favorable course (without metastases) of visceral cancer in Muir-Torrais’s syndrome and the importance of detecting tumors of the sebaceous glands in patients with keratoacanthoma are emphasized.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):9-11
pages 9-11 views

Norwegian scabies

Dubensky V.V., Fokina R.A., Arefyeva O.S., Kovbas I.S.


The data on the clinical course of Norwegian scabies, published in Russian and foreign journals, are reviewed. A clinical case is presented: male patient aged 29 years with the diagnosis of Norwegian scabies in-dicatеs the epidemiological significance of this disease. Examinations detected a complex of diseases: B23.8 stage 4A: relapsing bacterial infection, mucosal candidiasis, labial herpes, chronic hepatitis of presumably alcoholic origin, chronic bronchitis, chronic pyelonephritis, urolithiasis, thrombocytopenia.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):12-14
pages 12-14 views

Scabiotic erythroderma

Malyarchuk A.P., Sokolova T.V., Lopatina Y.V.


A new clinical form of scabies - scabiotic erythroderma - is described. Five cases with scabies, manifesting primarily by diffuse erythroderma, are presented. Diagnostic criteria for scabiotic erythroderma and differential diagnosis with Norwegian scabies are presented. The invasive potential of this disease form in the patient and beyond him/her is evaluated for the first time.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):15-22
pages 15-22 views

Chronic urticaria associated with primary biliary cirrhosis

Kolkhir P.V., Dzhavakhishvili I.S., Kochergin N.G., Nekrasova T.P., Rozina T.P.


Chronic urticaria (CU) is one of the 20 most prevalent cutaneous diseases. It is associated with spontaneous weals, itching, and/or angioedemas persisting 6 weeks or longer. The cause of CU remains obscure in 7090% cases, in the rest cases the disease can be due to concomitant diseases, including autoimmune ones. We offer one of the first descriptions of CU associated with a rare autoimmune disease - primary biliary cirrhosis (PBC) in a female patient aged 30 years. In recent time three cases with CU associated with PBC (including cases with normal level of hepatic enzymes) have been descibed, which may indicate a relationship between these two diseases. In our observation PBC was asymptomatic for a long time, but with concomitant urticaria and high levels of hepatic enzymes and specific autoantibodies. Our data indicate the need in more profound immunological diagnosis in some cases, even with slight CU, good quality of life, response to treatment, and without other symptoms of PBC..
Russian Journal of Skin and Venereal Diseases. 2014;17(3):22-25
pages 22-25 views

Biochemical aspects of steroid resistance of autoimmune pemphigus

Dukhanin A.S., Teplyuk N.P., Lepekhova A.A., Zemskova O.V.


The molecular mechanisms of steroid resistance in patients with autoimmune pemphigus (AP) were detected at the receptor level. Comparative analysis of the parameters of glucocorticoid specific binding by peripheral blood lymphocyte receptors and of the levels of glucocorticoid a- and в-receptors in the peripheral blood lymphocytes was carried out in patients with hormone-sensitive and hormone-resistant AP. Specific features of the clinical course of AP under conditions of changed levels of a - or в-receptors in steroid-resistant patients were detected.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):25-31
pages 25-31 views

Panniculitis caused by a 4-antitrypsin deficiency

Olisova O.Y., Grabovskaya O.V., Teplyuk N.P., Belousova T.A., Dzhavakhishvili I.S., Varshavsky V.A.


Presents data on panniculitis and its different forms with special emphasis on panniculitis caused by а 1-antitrypsin (а 1-AT) deficiency, difficulties in the diagnosis and treatment of this condition. The diagnosis ofа -AT deficiency is based on clinical laboratory studies (common analysis of blood, biochemical study, ultrasonic study, esophagogastroduodenoscopy, etc.), measurement of blood а -AT concentration, and molecular genetic studies - detection of PiZZ genotype (Glu342Lys mutation in homozygotic state). The results of authors ’ observations are presented: effective systemic glucocorticosteroid therapy of panniculitis caused by а -AT deficiency.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):32-35
pages 32-35 views

Difficulties in the diagnosis of systemic lupus erythematosus

Tlish M.M., Naatyzh Z.Y., Sycheva N.L., Boiko N.A., Irizelyan G.A.


Three clinical cases are presented: women with systemic lupus erythematosus (SLE), diagnosed after comprehensive examinations including histological studies of skin biopsy specimens and immunologic studies: blood testing for antinuclear factor on HEp-2 cells with evaluation of the nucleus fluorescence type, testing for plasma lupus anticoagulant, rheumatoid factor, antiphospholipid IgG and IgM, double-stranded DNA, and antinuclear auto-IgG to AMA-M2 (by the immunoblotting method). At first the disease clinically manifested only by skin involvement of the discoid lupus type on the face or head. SLE was diagnosed 1.5, 2, and 4 years after the skin involvement, in one case - disseminated lupus erythematosus in the presence of chronic nephritis, later classified as class IV lupus nephritis.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):36-41
pages 36-41 views

Studies of lichen planus pathomorphosis by evaluating its modern histological signs

Barbinov V.V., Chirsky V.S., Tarasenko G.N., Barbinov D.V.


Statistically significant histological models of lichen planus (LP) were detected by comparing histological preparations from 102 patients with the final diagnosis of LP, analyzed using a Histoderm expert system, with the final clinical diagnosis in this sample, after preliminary analysis (by the same expert system) of more than 600 histological symptoms vs. 224 other dermatoses presented in this system. Comparison of the results of two studies showed that of the symptoms determining various dermatoses, 49 were statistically significant for the diagnosis of LP, corresponding to its 6 histological models. Only 22 symptoms, characterizing only 2 of the probable histological models, were significant in skin biopsy specimens from our sample ofpatients with LP. The results indicated the LP pathomorphosis and indirectly confirmed the immunoallergic hypothesis of its pathogenesis.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):42-48
pages 42-48 views

Anxiety level and dermatology life quality index in patients with atopic dermatitis

Orlov E.V., Mostovaya L.I., Konnov P.E., Arsenyeva A.A.


Disorders in the psychoemotional sphere, associated with the majority of cutaneous disease, including atopic dermatitis, are responsible, among other things, for severe course of the disease and its drug resistance. Psychodiagnostic studies were carried out in 80 patients with atopic dermatitis. The examinations included clinical examination with evaluation of dermatological and psychological indexes and tests for evaluating the quality of life and severity of depressive and anxiety disorders in the patients. Specific features of the psychoemotional sphere of patients with atopic dermatitis were detected.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):48-51
pages 48-51 views

Complications presented by neuropathic disorders on the face after contour injection plasty

Mingazova L.R., Karpova E.I., Orlova O.R., Kotenko K.V., Kruglova L.S.


The injection methods of contour plastic reconstruction of soft tissues by means of gel, though effective, are not free from complications, which are presumably caused by the use of aggressive correction methods, causing injuries in the smallfibrils of the facial peripheral nerves. The causes of emergence and treatment of neuropathies, developing after contour injection plastic reconstruction (CIP) of the facial soft tissues (FST), receive little attention, and we therefore analyze the efficiency of combined therapy including laser exposure in this category of patients. The suggested algorithm of treatment ofpatients with neuropathies after FST CIP generally accelerates the rehabilitation of these patients.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):52-55
pages 52-55 views

Minolexin and its place in standard therapy for acne vulgaris

Snarskaya E.S., Minakova M.E., Kuznetsova E.V.


Propionebacteria acne react with congenital immunity markers - Toll-like (TLR) and protease-activated (PAR) receptors. In the epidermis TLR is expressed on epitheliocytes, keratinocytes, Langerhans ’ cells, macrophages, monocytes, and granulocytes, and is directly involved in the formation of antibacterial response. The role of PAR in the pathogenesis of acne consists in induction and stimulation of the unfolding inflammatory process via stimulation of expression of IL-1a, tumor necrosis factor-a, human b-defensin 2 (hBD2), matrix metalloproteinasesmMP-1, MMP-2, MMP-3, MMP-9, andMMP-13. Hence, P. acne is involved in many processes in the pathogenesis of acne vulgaris, including inflammation, hyperkeratosis, and excessive production of the sebum, this necessitating its eradication - an important component of combined therapy. Minolexin® (minocyclin) is a highly effective drug for therapy of medium-severe and severe forms of acne vulgaris. The drug is included in the European recommendations for therapy. The drug is characterized by a pronounced bacteriostatic effect and is highly lipophilic, rapidly penetrates through the bacterial lipid layer, and intensely cumulates in the sebaceous glands.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):55-59
pages 55-59 views

A case with tertiary active, early latent, and early congenital syphilis in one family

Serdyukova E.A., Rodin A.Y., Rusinov V.I.


A family of 3 patients with different forms of syphilis (congenital, early latent, and active tertiary) was observed. The disease was first detected in examination of the patients ’ daughter (2 months), hospitalized in intensive care wards of Regional Pediatric Clinical Hospital with the diagnosis of congenital hepatitis resistant to therapy. Blood testing for syphilis showed microprecipitation (MP) 4+, enzyme immunoassay (EIA) 4+. Early congenital syphilis (with visceral symptoms) was diagnosed. The parents were then examined. In the mother (23 years) no syphilis manifestations were found on the skin and mucosa. Laboratory findings: Wassermann’s test (WT) 4+, titer 1:320; MP 4+; EIA positive for summary antibodies (negative IgM, positive IgG, titer 1:160). Early latent syphilis was diagnosed. In the father (25 years) two foci of tubercular syphilis persisted during about 1 year. Blood analysis: WT 4+, 1:80; MP 4+; EIA positive for summary antibodies (negative IgM, positive IgG, titer 1:640). Tertiary active syphilis was diagnosed. The patients refused from spinal puncture. Specific therapy was carried out.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):60-62
pages 60-62 views
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Fistula with transparent discharge in the skin of the neck

Tarasenko G.N., Bobrov A.N., Chalyi S.N., Parkhomenko S.A., Demidov I.A.
Russian Journal of Skin and Venereal Diseases. 2014;17(3):64a
pages 64a views

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