Debut and course rare of juvenile dermatomyositis. Part I: clinical case
Keywords:
juvenile dermatomyositis, juvenile amyopathic dermatomyositis, atypical course, ulcerative necrotic glossitis, anasarca, psoriasis, treatmentAbstract
Describes the onset and course of a rare case of juvenile dermatomyositis. Its peculiar сlinical was the beginning of the disease with a predominance of signs of severe intoxication, ulcerative-necrotic damage of the tongue and anasarca. Typical cutaneous clinical manifestations of the disease were delayed by one month. Laboratory studies revealed an increase in the level of the acute inflammatory markers and titers of antinuclear antibodies, and among the enzymes specific for the myopathic syndrome, only increased alanine aminotransferase activity was positive. The electromyogram did not record any violations. Two years later, comorbidity of the disease with psoriasis vulgaris was noted. Long-term (seven-year) observation revealed chronic amyopathic course of the disease. It has been shown that the use of standard treatment with methylprednisolone, methotrexate and plaquenil in this case is not effective enough.
The second part of the article will be provide a review of the literature on other atypical variants of the onset and course of juvenile dermatomyositis and comparative clinical analysis of this clinical case with other similar observations.
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.
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