Vol 14, No 5 (2011)

Articles
Genodermatoses in works of V. A. Rakhmanov
Ivanov O.L., Mareeva E.B., Ivanov O.L., Mareeva E.B.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):4-6
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Perspectives of genotyping in dermatology
Lomonosov K.M., Lomonosov K.M.
Abstract
Authors present data about genotyping studies in dermatology illustrating the main applications and practical importance of genetic analyses for dermatologists.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):6-8
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Dermatological problems from a genetic point of view
Asanov A.Y., Asanov A.Y.
Abstract
Authors discuss influence of genetic factors on a clinical presentation and course of genetic dermatoses and multifactorial dermatoses, and underline the importance of technologies of molecular genetics for diagnosis and treatment of skin diseases.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):8-10
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Peculiarities of clinical presentation and therapy of different forms of Darrier disease
Kryazheva S.S., Snarskaya E.S., Kryazheva S.S., Snarskaya E.S.
Abstract
Literature data (45 Russian and foreign papers) as well own experience of treatment of different forms of Darrier disease in 12 patients. Peculiarities of the disease course and therapy are discussed.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):10-14
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Neurofibromatosis: the current state of the problem
Asanov A.Y., Filippova M.G., Asanov A.Y., Filippova M.G.
Abstract
Neurofibromatosis is a genetically heterogenic group of monogenic diseases with determined etiology (neurofibromatosis I, neurofibromatosis II, schwannomatosis) and disorders with unclear molecular nature. In this review diagnostic criteria of neurofibromatosis I and II and schwannomatosis are presented. New data concerning correlation of genotype and phenotype in neurofibromatosis I as well as clinical and genetic characteristics of other forms.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):14-20
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Metabolic syndrome and porphyria cutanea tarda
Krivosheev A.B., Krivosheev B.N., Kondratova M.A., Krivosheev A.B., Krivosheev B.N., Kondratova M.A.
Abstract
Authors carried out a comparative assessment of comorbid metabolic syndrome (MS) on a clinical course, severity of disorders of porphyrin, lipid, and carbohydrate metabolism in patients with porphyria cutanea tarda (PCT). Sample included 34 patients (31 men and 3 women) from 33 to 68 years old (mean age 48 ± 2,1 y.o.). In 20 patients (main group) MS was diagnosed which was associated with hepatitis C virus infection (HCV). The comparison group included 14 patients without any signs of MS. The examination revealed no significant influence of MS on a clinical course and dermatological manifestation of PCT. But in patients with MS cardiovascular disorders were more often present, as well as disorders of lipid and carbohydrate metabolism along with higher levels of uroporphyrin in urine. The additive effect of MS and HCV infection on a liver function is unfavourable. As a rule, patients with PCT and MS have comorbid polyorgan pathology and should be treated as patients with higher risk of coronary complications and diabetes mellitus II.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):20-27
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A case of Naegelli's pigmented reticular dermatosis comorbid to Futcher's pigmented demarcation line
Kryazheva S.S., Snarskaya E.S., Belova N.I., Kryazheva S.S., Snarskaya E.S., Belova N.I.
Abstract
Analysis of literature data is presented concerning clinical picture of Naegelli's pigmented reticular dermatosis and Futcher's pigmented demarcation line. A case of combination of these disorders in a child is described.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):27-29
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Broadening the possibilities of topical glucocorticosteroids use in treatment of patients with atopic dermatitis
Keniksfest Y.V., Grishaeva E.V., Gorbunov A.P., Keniksfest Y.V., Grishaeva E.V., Gorbunov A.P.
Abstract
In order to increase efficiency and safety of treatment of atopic dermatitis among children and adults modern glucocorticosteroid ointment methylprednisolone aceponate is offered for drug phonophoresis method.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):29-31
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A case of Muir-Torre syndrome
Molochkov V.A., Kuntsevich Z.S., Khlebnikova A.N., Vlasova M.A., Bochkareva E.V., Lavrov D.B., Molochkov V.A., Kuncevich Z.S., Khlebnikova A.N., Vlasova M.A., Bochkareva E.V., Lavrov D.B.
Abstract
A case of Muir-Torre syndrome is described, which manifested with a combination of skin tumors (keratoakantoma, adenoms or cancer of sebaceous glands and neoplasia of internal organs). Most often gastrointestinal tract and urogenital system are affected. Authors present a case of persistent giant keratoacantoma, sebaceous adenomas and prostate adenocarcinoma in a patient. Sebaceous tumors are the obligatory marker of Muir-Torre syndrome, that is why it is important to look for internal neoplasia after the detection of these tumours.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):31-33
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Congenital dystrophic hyperplastic bullous epidermolysis of Cockayne-Touraine
Snarskaya E.S., Kryazheva S.S., Kartashova M.G., Bobrov M.A., Filatova I.V., Snarskaya E.S., Kryazheva S.S., Kartashova M.G., Bobrov M.A., Filatova I.V.
Abstract
Authors present a review of russian and foreign literature (22 papers) concerning etiology, pathogenesis, classification, diagnosis and treatment of bullous epidermolysis. A case of congenital dystrophic bullous epidermolysis in a 15 years old patient is described.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):34-41
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Problems of physical development of patients with bullous epidermolysis
Bobko S.I., Al'banova V.I., Bobko S.I., Albanova V.I.
Abstract
The data are presented demonstrating an apparent delay in physical development in patients with bullous epidermolysis: at birth the stature and weigh correspond to norms, and during the further growth significant delay in physical development becomes apparent. The reasons of this disorder are discussed as well as appropriate nourishment measurements.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):42-43
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Gronblad-Strandberg syndrome
Kryazheva S.S., Snarskaya E.S., Kartashova M.G., Filatova I.V., Kryazheva S.S., Snarskaya E.S., Kartashova M.G., Filatova I.V.
Abstract
Etiology, pathogenesis, clinical and histopathologic diagnostic criteria as well as therapeutic approach to Gronblad-Strandberg syndrome are discussed. A case of Gronblad-Strandberg syndrome is presented. The disorder manifested in 47 years old patient with disseminated pseudoxantoma elasticum on a skin of neck, axillae, and other zones as well as angioid stripes in Bruch's membrane and lower legs arteries affection.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):43-47
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Inherited scleroderma
Snarskaya E.S., Romashkina A.S., Snarskaya E.S., Romashkina A.S.
Abstract
Review of Russian and foreign literature (18 papers) concerning inherited scleroderma is presented in two and three generations of relatives.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):47-50
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Two cases of inherited ichthyosis
Serdyukova E.A., Rodin A.Y., Serdyukova E.A., Rodin A.Y.
Abstract
Authors present two cases of such rare disorder as inherited ichthyosis in newborns, successfully treated in in-patient department. Current literature data concerning etiology, pathogenesis, clinical presentation and therapy of inherited ichthyosis are also presented.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):50-52
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Monilethrix
Tikhonovskaya I.V., Kozlovskaya V.V., Tikhonovskaya I.V., Kozlovskaya V.V.
Abstract
Monilethrix - rare genetic disorder of hair shaft with clinical picture of hair fragility. The disease is inherited autosome-dominantly and develops as a result of disorder of keratin synthesis. Review of thw literature and case report are presented.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):52-54
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Genodermatoses in Primorye territory: clinical heterogeneity of Werner syndrome
Yutskovskiy A.D., Yutskovskaya Y.A., Stupin A.V., Fedorova E.B., Kulagina L.M., Koval'chuk E.V., Yutskovskiy A.D., Yutskovskaya Y.A., Stupin A.V., Fedorova E.B., Kulagina L.M., Kovalchuk E.V.
Abstract
Genodermatoses - a problem, which needs more detailed investigations and organization of patients rehabilitation. During the last 15 years in Primorye territory 58 cases of genodermatoses were detected. Ar the same time genodermatoses are still rarely timely diagnosed because of heterogeneity of clinical presentation. Besides that, as a rule these patients stay for follow up by non-dermatologists. Obviously such situation is a result of insufficient knowledge in this field and luck of communication between detmatologists and other specialists, shortage of guidance materials and guidelines on genodermatoses for practicing specialists of health service. Several cases of Werner syndrome and inherited psoriasis are presented.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):55-57
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Calcineurin inhibitors in atopic dermatitis
Bilalova U.G., Kochergin N.G., Lytkina E.V., Mel'nichenko A.B., Potekaev N.N., Smirnova L.M., Chernikova E.A., Bilalova U.G., Kochergin N.G., Lytkina E.V., Milnichenko A.B., Potekayev N.N., Smirnova L.M., Chernikova E.A.
Abstract
Authors discuss the influence of phenotipic heterogeneity in atopic dermatitis on therapeutic effectiveness of different drugs. As an example a group of calcineurin inhibitors (ciclosporin A, pimecrolimus and tacrolimus). Some results of international and own studies are presented.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):58-62
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Clinical effectiveness of "Aysida" preparations - cream-gel for dry and sensitive skin
Shperling N.V., Vengerovskiy A.I., Shperling I.A., Shperling N.V., Vengerovskiy A.I., Shperling I.A.
Abstract
For the purpose of research of agents for treatment of skin diseases, accompanied by skin dryness, an assessment of clinical effectivity of "Aysida" preparations (cream-gel for dry and sensitive skin). The cream-gel was applied in patients after the main treatment course for moderate to severe atopic dermatitis (AD), and as main treatment preparation for mild AD and skin xerosis
Russian Journal of Skin and Venereal Diseases. 2011;14(5):62-64
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Artificial stigmates
Monakhov S.A., Monakhov S.A.
Russian Journal of Skin and Venereal Diseases. 2011;14(5):65-65
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