Debut and course rare of juvenile dermatomyositis. Part II: clinical analysis with comparative analytical review of the literature

Abstract

The first part of the article describes a rare case of the onset and course of juvenile dermatomyositis. In the second part the comparative clinical analysis of results of own supervision with other similar reports is carried out. The main attention is focused on atypical variants of the onset of the disease, in particular signs of severe intoxication syndrome, hemorrhagic-necrotic rash, anasarca, lesions of the oral cavity in the form of stomatitis and ulcerative-necrotic glossitis. The difficulties of early diagnosis of the disease on the background of delayed manifestation of pathognomonic skin signs and the absence of reliable features of myopathic syndrome are shown. Clinical and laboratory characteristics of amyopathic juvenile dermatomyositis with persistent skin lesions have been defined. There are three differentiated plans-recommendations of the Childhood Arthritis and Rheumatology Research Alliance for the treatment of patients who have never had muscle damage and those who have had clinical manifestations of myopathy only in the first months of the disease. Current trends in achievements and gaps in the classification, diagnosis and treatment of various forms of juvenile dermatomyositis have been observed. On the basis of scientific publications the possible prognostic consequences of the amyopathic form of this disease in children are highlighted. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: juvenile dermatomyositis, juvenile amyopathic dermatomyositis, atypical course, ulcerative necrotic glossitis, anasarca, psoriasis, treatment.