An unexpected presentation of Langerhan’s Cell Histiocytosis

Docherty, N. M. E., Mather, C. I. and Bell, A. (2015) An unexpected presentation of Langerhan’s Cell Histiocytosis. Oral Surgery, 8(2), pp. 11-114. (doi: 10.1111/ors.12156)

Abstract

Langerhan's Cell Histiocytosis (LCH) refers collectively to a group of diseases previously known as Histiocytosis X, eosinophilic granuloma, Abt-Letterer-Siwe syndrome, Hand-Schueller-Christian syndrome, and others. LCH is rare with an estimated annual incidence of 1–7 cases per million of the population. It has a 2:1 preponderance for presentation in males, usually in childhood, however literature suggests increasing numbers of later presentations. The aetiology of LCH is unknown though there is evidence to suggest that LCH may be a more neoplastic disease than a reactive disorder but pathogenesis is still unclear. The disease can affect any organ or system but more frequently bones, skin and pituitary gland. Skeletal involvement is one of the most common features with a reported incidence in the jaws of 7.9%. Involvement of the maxillary sinus is rare. Diverse clinical presentation can lead to outcomes ranging from spontaneous remission of a single bony lesion to rapidly progressive systemic disease that leads to multi organ failure and death. We report an unexpected presentation of LCH in a 34 year old male presenting with a large oro-antral fistula (OAF) following extraction of the upper right first molar some months previously. The OAF was successfully repaired using a buccal fat pad/advancement flap. At time of surgery an unusually large volume of bone loss was noted extending to the apices of adjacent teeth, with what appeared to be chronic granulation tissue at the base of the socket extending into the maxillary sinus. This tissue was sampled and histopathology confirmed a diagnosis of LCH. The post-operative recovery was uneventful, but given the diagnosis the patient was sent for further assessment by our haematology colleagues. This report reviews current literature and highlights the importance of sampling tissue in seemingly routine procedures particularly when little is known of the history of the presenting clinical complaint.

• Item Type: Articles
• Additional Information: British Association of Oral Surgeons 2014 Open Papers
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Bell, Professor Aileen and Mather, Mr Craig and Docherty, Mrs Nicola
• Authors: Docherty, N. M. E., Mather, C. I., and Bell, A.
• Subjects: R Medicine > RD Surgery
• College/School: College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing > Dental School
• Journal Name: Oral Surgery
• Publisher: Wiley
• ISSN: 1752-2471
• ISSN (Online): 1752-248X
• Published Online: 23 April 2015