Histiocytose congénitale auto-involutive de Hashimoto-Pritzker.

Translated title of the contribution: Hashimoto-Pritzker congenital self-healing histiocytosis

F. Cambazard, J. Kanitakis, G. Zambruno, P. Barre, D. Jacquemier, J. Thivolet

Research output: Contribution to journalArticle

Abstract

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.

Translated title of the contributionHashimoto-Pritzker congenital self-healing histiocytosis
Original languageFrench
Pages (from-to)213-217
Number of pages5
JournalPediatrie
Volume43
Issue number3
Publication statusPublished - 1988

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Cambazard, F., Kanitakis, J., Zambruno, G., Barre, P., Jacquemier, D., & Thivolet, J. (1988). Histiocytose congénitale auto-involutive de Hashimoto-Pritzker. Pediatrie, 43(3), 213-217.