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Langerhans cell histiocytosis: a diagnostic dilemma

From Volume 39, Issue 10, December 2012 | Pages 716-720

Authors

Paul L Ryan

BDS, MFDS RCS(Eng), MClinDent

Academic Clinical Fellow (Specialist Registrar) in Restorative Dentistry, Institute of Dentistry, Barts and The London School of Medicine and Dentistry, Turner Street, London

Articles by Paul L Ryan

Kim M Piper

BDS, FDS RCS(Eng), PhD, MA, FRCPath

Honorary Consultant in Oral Pathology, Oral Pathology, Dental School Division, Turner Street, London

Articles by Kim M Piper

Francis J Hughes

BDS, PhD, FDS RCS, MBE, BDS, PhD FDS RCS Eng

Professor of Periodontology, Dental Institute, King's College London, Floor 21 Tower Wing, Guy's Hospital, Great Maze Pond, London SE1 9RT, UK

Articles by Francis J Hughes

Abstract

Langerhans cell histiocytosis (LCH) is a rare clonal neoplastic disorder of unknown aetiology which can present with a diverse range of clinical presentations. It encompasses a diverse number of idiopathic conditions which can involve multiple body systems and is characterized by bone marrow-derived Langerhans cell proliferation. The disease can affect multiple body systems and lesions can be solitary or widespread. We present a case of a multifocal eosinophilic granuloma (LCH) in a young adult female with clinical signs and symptoms similar to aggressive periodontitis.

Clinical Relevance: Langerhans cell histiocytosis is a rare disease which can have a similar clinical presentation to aggressive periodontitis.

Article

Langerhans cell histiocytosis encompasses a diverse number of idiopathic conditions which can involve multiple body systems.1 It is characterized by bone marrow-derived Langerhans cell proliferation and can affect many body systems, including: bone, lung, skin, mucous membranes, lymph nodes and the liver. The lesions can be solitary or widespread.

LCH includes a group of disorders which are classically described as being of one of three forms with distinct clinical presentations. The eosinophilic granuloma normally presents as a solitary slowly progressing lesion in the bone, lungs or stomach, often found as an incidental radiographic finding typically in young adults. It tends to be more benign in nature and solitary, responding well to conservative local treatment. Letterer-Siwe disease, in contrast, is a multi-system rapidly progressing form of the condition in very young children, which is treated with chemotherapy, but has a very poor survival rate. The final condition which makes up the disease triad is Hand-Schüller-Christian disease which is a multifocal eosinophilic granuloma with multiple bony lesions, exophthalmos and diabetes insipidus. This typically affects young children, on average between 2–5 years, and lies somewhere intermediate to the other forms of LCH in terms of prognosis and severity. Disease onset can occur several years before symptoms and signs lead to a diagnosis.

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