Langerhans cell histiocytosis with oral manifestations: a rare and unusual case report

Abstract

Langerhans cell histiocytosis (LCH), is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. We present a case of a 32 years old, completely edentulous female patient who presented with erythema of hard palate, maxillary alveolar mucosa and mucosa over the distobuccal part of mandibular alveolar ridge with foci of ulcerations. Histopathologic features were suggestive of LCH which was confirmed by immunohistochemistry which was CD1a positive, confirmatory for LCH. Bone scan revealed multiple bone involvement. At this stage, disease had already progressed to multisystem involvement with endocrinal abnormalities (primary hypothyroidism and hyperprolactinemia), requiring aggressive treatment. Therefore, this case is a reminder of the possibility of occurrence of this rare disease in the oral cavity which might manifest itself in multiple presentations thus easily leading to the misdiagnosis and therefore, it could be easily overlooked by dentists. Key words:Langerhans cell histiocytosis, immunohistochemistry, bone scan.

Langerhans cell histiocytosis revisited: Case report with review

“…The age of onset varies depending upon the variety of LCH and are rarely seen in adults with incidence around 1–2 cases/million persons per year. [ 4 ] The present case was reported in adult male of 29-year-old. Solitary or multiple bone lesions are the most common clinical presentations seen in the majority of cases, with a most frequent sites involving skull, ribs, vertebrae, and jaws.…”

“…Hashimoto Pritzker syndrome is a congenital form of LCH presenting with a deep subcutaneous skin lesions. [ 4 6 ] Solitary type of eosinophilic granuloma is twice as common as multiple variants and is most common among other three variants. In the present case, the patient exhibited a solitary skeletal lesion with no extraskeletal involvement hence was considered as eosinophilic granuloma variant.…”

Rare Langerhans cell histiocytosis in children: a case report

“…However, recent literature has shown monoclonal proliferation of lesional cells, which is suggestive of neoplastic lesion. [10][11][12][13] The predilection of LCH in males is twice that it is in females with LCH affecting children between the age 1 and 15 with the peak between 2-4 years old14 yearly incidence of children aging <10 years old is thought to be 1 in 200,000 the reported case is in an 11-year-old male child. 14 In the majority of cases, LCH most common clinical signs are bone lesions that are either solitary or multiple involving ribs, vertebrae, skull and jaw.…”

A rare and unusual case report of Langerhans cell histiocytosis

“…[6] Among children under the age of 10 years, yearly incidence is thought to be 1 in 200,000. [7] The present case was reported in a 3-year-old male child.…”