Langerhans' cell disease: literature review

Abstract

The authors present a literatu- re review on Langerhans' cell di- sease, also known as idiopathic histiocytosis and previously na- med histiocytosis X. The disease is manifested in three distinct clinical forms: a) eosinophilic gra- nuloma, the most frequent and mildest manifestation, considered the localized chronic form; b) Hand-Schüller-Christian syndro- me, corresponding to the dissemi- nated chronic form with a triad of symptoms which includes exo- phthalmos, diabetes insipidus and osteolytic skull lesions; c) Lette- rer-Siwe syndrome, representing the disseminated acute form whi- ch affects infants and newborns. Although Langerhans' cell disea- se is an unusual entity, its clinical and radiographic features may be confused with highly common lesions in dental clinics such as periapical granulomas, periapical cysts and periodontal diseases. Since oral manifestations are fre- quently observed in this disease, the dentist must know its featu- res and include it in the differen- tial diagnosis of periodontal and periapicallesions. Key words: Langerhans' cell disea- se, idiopathic histiocytosis, eosi- nophilic granuloma, Hand-Schül- ler-Christian syndrome, Letterer- Siwe syndrome.