Diffuse fasciitis with eosinophilia in childhood.

Abstract

Diffuse fasciitis with eosinophilia is a newly recognized connective tissue disease appearing initially to be either scleroderma or dermatomyositis. It has clinical and histologic features that allow it to be clearly separated from both disorders and has a more favorable prognosis. The majority of reported cases have been in adults. The disease involves the extremities, and at times the trunk, diffusely. One sees firm, often puckered or rivuleted skin, tightly bound to underlying structures; contractures result in several weeks. Early peripheral blood features are eosinophilia and elevated immunoglobulins. For diagnosis, full-thickness biopsies extending from skin through subcutaneous tissue, fascia, and muscle are taken together as one block. These tissues should not be separated from each other. The diagnostic lesion is in the fascia which is edematous, thickened, and infiltrated by mononuclear cells. The other tissues are completely normal. The clinical response to steroids is prompt and favorable.