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Twenty-six cases of dermatomyositis in children with follow-up studies ranging from one to 19 years are presented in order to indicate the natural history of this disease. Of these 26 children, 10 are dead, 4 still have active disease, 4 have crippling contractures and 8 are able to lead an active normal life. Thirteen of the children received endocrine therapy with adrenocorticotropic hormone, cortisone or testosterone. This appeared to produce at least symptomatic benefit in some cases, particularly early in the course of the active disease and, coordinated with symptomatic care, helped to tide the patients through the acute stages. The most important treatment consists of persistent orthopedic measures and physiotherapy to minimize contractures. Since death in this disease often results from palato-respiratory involvement, maintenance of an adequate airway and the use of a mechanical respirator may be life-saving.
Wedgwood et al. (Thu,) studied this question.