[Hashimoto-Pritzker congenital self-healing histiocytosis]

A full-term female infant was born with numerous purple firm indolent papulonodular lesions, occasionally ulcerated, ranging in size from 1 to 8 mm, scattered all over the body. The liver, spleen, lymph nodes were normal, as were the blood count, skull and chest X-ray. Biopsy of a skin lesion showed a deep dermal infiltrate consisting of histiocytic cells which were OKT6 +, HLA-DR + and S 100 +. Electron microscopy revealed numerous dense bodies, often with a myelin-like core and some Birbeck granules. The clinical course was favourable with a spontaneous regression of all lesions, sometimes through a central necrosis or atrophy, within 8 weeks. Congenital self-healing reticulohistiocytosis (CSHR) is characterized by a neonatal onset, with purple or necrotic nodular lesions showing the histopathologic aspect of histiocytosis X (although Birbeck granules are less numerous), an absence of visceral involvement and a spontaneous regression within the first 3 months of life. The place of CSHR among histiocytosis is discussed.