Original paper
Scleromyositis in children – diagnostic difficulties

.







Scleromyositis is an overlap syndrome of scleroderma and poly/ dermatomyositis with the immunological marker: the PM-Scl antibody detected by indirect immunofluorescence on Hep-2 cells. The aim of our study was to identify specific clinical symptoms for an initial period of the disease and to determine the performance of certain tests at the beginning of the disease and its course, which would be useful in fixing the diagnosis at beginning the treatment. Analyzed group consisted of 33 children (11 boys and 22 girls). We assessed joints, muscles, skin: the specific changes of SSc and DM, Raynaud phenomenon, and also changes in the internal organs. Based on this clinical analysis the following results have been achieved: • A child with the following symptoms: – Scleroderma-like changes of face and hands, Gottron’s sign, periorbiatal erytema, psoriasis-like changes of knees and elbows, – muscle atrophy and weakness or myalgia, – arthralgia or arthritis, or progressively reducing movement especially the hand’s joints, – Raynaud’s phenomenon, require the observation in the direction of scleromyositis and implementation of supplementary tests. • Children, who at the beginning of the disease posses just some of the characteristic symptoms of this syndrome require observation for a period of 3 years in order to determine the proper diagnosis.