Introduction
Langerhans cell histiocytosis (LCH) is a rare hematological disorder
characterized by intense and abnormal proliferation of bone
marrow-derived immature myeloid dendritic cells; Langerhans cells (LCs)
in the skin, bone, lymph nodes, and other organs.1Although, due to its low occurrence, limited information on the
epidemiology of LCH is available, an estimated 5 to 9 cases per million
in children < 15 years of age, and 1 case per million in
patients > 15 years of age have been
reported.2
Depending on the different clinical manifestations, LCH had been
classified conventionally by the International Histiocyte Society into
three different types: Type I - Eosinophilic granuloma (chronic focal
LCH), Type II - Hand-Schüller-Christian disease (chronic disseminated
LCH) and Type III - Letterer-Siwe disease (acute disseminated
LCH).3 However, because clinical findings are widely
manifested, the disease has also been classified into unifocal and
multifocal forms; depending on the extent of its spread throughout the
body. The unifocal form presents only one destructive lesion in an
organ, affecting bones or skin (80% of cases), liver, spleen, brain,
and lymph nodes more frequently3 The disease manifests
itself in a variety of ways, from isolated disease that resolves on its
own to life-threatening multisystem disease, with a 20% mortality
rate.4
The involvement of head and neck region is quite common in LCH,
particularly the bones of skull and jaws. The oral soft tissue lesions
are also common and are mostly seen on the hard palate and gingiva.
Since the symptoms of LCH may first appear in the oral cavity before
occurring elsewhere in the body, a thorough knowledge of the clinical
presentation of the disease is important.1
Awareness among clinicians regarding the clinical, histological, and
immune-histopathological features of the disease will help in early
diagnosis and treatment for better prognosis. Thus, this report aims to
present a case of Langerhans cell histiocytosis with the oral and dermal
manifestations seen in a pediatric patient.