Juvenile overlap syndrome: Considering scleromyositis

Abstract

Scleromyositis refers to a combination of characteristics found in both systemic scleroderma and polymyositis, with identification often based on the presence of the PM-SCL antibody. Currently, there are no well-defined criteria for classifying this syndrome. In childhood cases exhibiting sclerodermoid features, they are often categorized as scleroderma due to the distinct nature of the condition in this age group. We report two cases of children presenting with cutaneous signs of systemic scleroderma and associated myositis. The first case exhibits sclerodactyly, calcinosis, and myalgias with a positive anti-PM/SCL antibody, while the second case shows swollen fingers, clear Raynaud’s phenomenon, and myalgias but a negative anti-PM/SCL antibody. The clinical and biological variation in these cases prompts the dermatologist to consider scleromyositis as a juvenile overlap syndrome, given the sclerodermiform pattern and myositis, regardless of the positivity or negativity of the anti-PM/SCL antibody. As for the treatment, managing Raynaud’s and initiating a low dose of corticosteroid therapy, possibly combined with immunoglobulins, appears to be effective.