Severe course of disabling pansclerotic morphea of childhood: clinical features and therapy

Disabling pansclerotic morphea of childhood is the most severe variant of l ocalized scleroderma. It is characterized by rapid progression of deep cuta neous fibrosis expanding over large areas of body surface. The prognosis in terms of normal life activity is poor and the disease may even take a fata l course. Presented is a case with extremely severe and rapidly progressive lesions resulting in cutaneous ulcerations joint contractures,and multilai ng deformities of the extremities. Histopathological analysis revealed exte nsive intravascular calcinosis of the small vessels, which may be an import ant factor in the pathogenesis of this poorly understood disease. UVA(1)-ph ototherapy was performed and induced a softening of sclerosis and a distinc t decrease of skin thickness. Based on these observations UVA(1)-photothera py may be promising in the treatment of extensive sclerotic disease of this kind, and possibly other diseases accompanied by excessive sclerosis.