A very unusual clinical case of histiocytosis X in a young girl with no unusual medical history, is presented. The patient presented with chronic suppuration of the groin and complex anal fistulae and multiple recurrent abscesses, despite surgical treatment (abscess drainage, terminal colostomy). Intestinal and immunologic evaluation were normal. Only histology made the diagnosis. Chemotherapy (vinblastine) resulted in healing and the colostomy was closed.
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