Abstract
This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach.
Highlights
Langerhans cell histiocytosis (LCH), formerly histiocytosis X, is a rare entity which may appear under 3 distinctive clinical forms, namely eosinophilic granuloma Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome [1]
LCH is a rare disorder that occurs at all ages but predominantly affects children and young adults
To make a clinical diagnosis of LCH when its lesions are restricted to oral mucosa usually is very difficult since only few cases have been reported on the literature [6 - 9], producing limited information of this uncommon manifestation of this disease
Summary
Langerhans cell histiocytosis (LCH), formerly histiocytosis X, is a rare entity which may appear under 3 distinctive clinical forms, namely eosinophilic granuloma Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome [1]. Eosinophilic granuloma represents the most common form of LCH and affects older children and adults, most patients being younger than 20 years of age. Oral manifestations are present in most cases and mainly include jaw lesions affecting the alveolar bone, leading to dental mobility and premature tooth loss [4].
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