Regressing atypical histiocytosis:A cutaneous proliferation of atypical neoplastic histiocytes with unexpectedly indolent biologic behavior

Abstract

Regressing atypical histiocytosis (RAH) of skin is a cutaneous noduloulcerative proliferation of atypical neoplastic histiocytes with concomitant polymorphous inflammation, frequently pronounced epidermal hyperplasia, and an unexpectedly indolent biologic course. Spontaneous regression and recurrence without systemic spread were the course in follow-up periods of over six years. Histopathologically, characteristic-appearing atypical mononuclear and multinucleated "RAH" cells showed erythrophagocytosis as well as ultrastructural, surface marker, and enzyme cytochemical features indicating histiocytic differentiation. Cytogenetic analysis showed aneuploidy and several marker chromosomes including 14q+. Its benign biologic course clearly distinguished this entity from malignant histiocytosis, large cell lymphoma, and Hodgkin's disease. The histiocytic atypical cells further distinguished it from the T-cell lesions of the skin, such as mycosis fungoides and lymphomatoid papulosis. This entity is readily confused with malignant lymphoreticular disease, melanoma, or squamous carcinoma.