Disabling pansclerotic morphea of children

Fourteen children with generalized morphea involving all levels of the skin and soft tissues were examined. The term "acral pansclerotic morphea" describes the distribution and the multiple levels of sclerosis. Lymphocytic inflammation and hyaline pannicultitis were observed on biopsy specimens in some cases. Laboratory data were characterized by a polyclonal elevation of gamma-globulin level and by peripheral eosinophilia. Pulmonary changes in five patients and esophageal changes in one imply that acral pansclerotic morphea may be assoicated with mild nonprogressive visceral change. Although cyclophosphamide may retard the process, no satisfactory treatment for progressive, mutilating acral pansclerotic morphea has been found.