Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report

Abstract

BackgroundLangerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects – by order of decreasing frequency – the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults.Case presentationWe describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen.ConclusionsGastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report.Electronic supplementary materialThe online version of this article (doi:10.1186/s13256-017-1428-7) contains supplementary material, which is available to authorized users.

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis

“…Although the disease is usually encountered in patients between 1 and 10 years of age with male predominance, recently reported cases described LCH in a five‐month‐old infant and patients aged between 17 and 63 years old. The present article discusses the case of a 2‐year‐old male …”

“…The present article discusses the case of a 2-year-old male. 2,7,9,12,14,15,18 The disease may be present with solitary or multiple osteolytic lesions in single or multiple bones with the head and neck being the most commonly affected, particularly the jaws. 17 Oral manifestations may include gingivitis, periodontitis, tooth rotation or loss, and malocclusion.…”

“…Langerhans cell histiocytosis, previously known as histiocytosis X, is a rare and destructive disease that may clinically and radiologically mimic malignant disorders . A high index of suspicion is needed to consider the diagnosis of LCH particularly in pediatric patients with unclear osteolytic lesions.…”

“…The combination of the rarity of the disease (it affects fewer than five people per million), the diversity of its clinical manifestations, and the need for histopathological diagnosis lead to difficulty in diagnosis . The histiocytes may infiltrate and damage single or multiple organs particularly bones . LCH occurs most commonly in the head and neck region, and when jaws and gingiva are affected, the patients may have nonspecific symptoms and signs such as gingival necrosis and hypermobility of the teeth (floating teeth) that may lead to misdiagnosis .…”

“…[1][2][3][5][6][7] The histiocytes may infiltrate and damage single or multiple organs particularly bones. [8][9][10][11][12] LCH occurs most commonly in the head and neck region, and when jaws and gingiva are affected, the patients may have nonspecific symptoms and signs such as gingival necrosis and hypermobility of the teeth (floating teeth) that may lead to misdiagnosis. 4,7,[13][14][15][16][17] This article discusses the oral manifestations of LCH, radiological findings, histological features, review of literature, and the role of dentists in the diagnosis.…”