PERIO - Periodontal Practice Today
PERIO - Periodontal Practice Today 5 (2008), No. 4     4. June 2009
PERIO - Periodontal Practice Today 3 (2006), No. 1  (16.03.2006)

Page 37-42

Hand-Schüller-Christian Syndrome (Disease) - Multiple Eosinophilic Granulomas in the Maxillary Bones Compromising the Periodontal Tissues: Case Report
Hidrobo, Rodrigo/Vargas, Alex/Martínez, Benjamín/Meyle, Jörg
We report a clinical case of a three-and-a-half-year-old girl with multiple radiolucent central lesions within the body and the ascending ramus of the mandible and the alveolar and basal bone of the maxillary sinus. In both maxillary bones additional destruction of the periodontal tissues was seen from the molar region to the canines. We confirmed a definitive diagnosis by histological analysis of a Langerhans cells histiocytosis (histiocytosis X) in its sub-variant of Hand-Schüller-Christian (H-S-C) syndrome (co-existence of diabetes insipidus and desquamative skin injuries). This case report illustrates the potentially rapid growth of eosinophilic granulomata, the difficulties and importance of a prompt diagnosis, and secondary periodontal infections with periodontopathogenic micro-organisms.

Keywords: histiocytosis X, eosinophilic granuloma, periodontal disease