Recurrent histiocytosis X as solitary mandibular lesion (case report on a 54 yrs old woman); [Histiocitosi X recidivante con localizzazione mandibolare]

Abstract

Histicytosis X is the generic term for a group of three diseases having similar clinical features: eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Sive disease. These diseases occurr mostly in children and young adults and progress rapidly with fatal spreading from a solitary skin or soft tissue lesion. Primary lesions affecting the head and neck usually carry the most severe prognosis. We present the case of a 54 yrs old patient who presented with histicytosis X in the form of a solitary mandibular lesion. The patient initially underwent surgical treatment by curettage, as indicated by protocols, but presented with local recurrence a year later. Ample resection of the mandible was then undertaken and reconstruction was achieved by an iliac autograft. Clinical and radiological check-ups show no evidence of local or systemic recurrence after a period of three years.