Variant of Congenital Self-healing liceloMstiocytosis: Solitary HasMmoto-Pritzker Disease
نویسندگان
چکیده
Four neonates had solitary, congenital, rapidly growing, spontaneously ulcerating tumors of the face, trunk, and extremities. No extracutaneous involvement was found, and all lesions spontaneously involuted. Mononuclear cells of the cutaneous infiltrate were Langerhans' cells. These findings expand the spectrum of congenital self-healing reticulohistiocytosis. The Langerhans' cells (LC), dendritic cells primarily located in the epidermis and some other epitheiia, can be regarded as members of the mononuclear-phagocyte system. One of their functions is to present selected antigens to T lymphocytes (1,2). Disorders of Langerhans' cells have been difficult to study, since LC are not specifically identifiable in routine histologic material. Histiocytosis X (3) in all its forms and congenital self-healing reticulohistiocytosis (CSHR) (4-10) are the only two diseases identified to date as proliferations of the cells. Cutaneous lesions in both conditions usually are multiple. We observed four neonates with solitary, ulcerating, congenital tumors containing Langerhans' cells that subsequently have followed a benign course.
منابع مشابه
Solitary, late-onset, self-healing Langerhans cell histiocytosis.
© 2011 The Authors. doi: 10.2340/00015555-0990 Journal Compilation © 2011 Acta Dermato-Venereologica. ISSN 0001-5555 “Self-healing” Langerhans’ cell histiocytosis (LCH), first reported by Hashimoto & Pritzker in 1973 (1), is a rare primary skin disorder that presents at birth or just after birth. It can be categorized based on the involvement of solitary or multiple sites and can be further cla...
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