CHILDHOOD SCLERODERMATOMYOSITIS - REPORT OF A CASE WITH THE ANTI-PM SCL ANTIBODY AND MECHANICS HANDS/

We report a boy with overlap manifestations of systemic sclerosis and dermatomyositis (sclerodermatomyositis) whose disease showed a changin g clinical pattern, and who had mechanic's hands, which are a cutaneou s marker of myositis, Serological studies revealed antinuclear antibod ies with a homogeneous nucleolar pattern. The anti-PM-Scl antibody was demonstrated by immunoblotting, HLA typing was positive for HLA-DR3/4 . After a follow-up period of 11 years, no progression to severe syste mic involvement was detected, and aggressive treatment was not adminis tered. The recognition of subsets of patients with homogeneous clinica l features and serological markers should permit the recognition of se parate conditions among overlap syndromes, This would have prognostic and therapeutic implications.