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Adult‐onset systemic L angerhans cell histiocytosis mimicking inflammatory bowel disease: the value of skin biopsy and review of cases of L angerhans cell histiocytosis with cutaneous involvement seen at the M ayo C linic
Author(s) -
Podjasek Joshua O.,
Loftus Conor G.,
Smyrk Thomas C.,
Wieland Carilyn N.
Publication year - 2014
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4632.2012.05716.x
Subject(s) - medicine , gastrointestinal tract , systemic disease , biopsy , skin biopsy , histiocytosis , langerhans cell histiocytosis , inflammatory bowel disease , diarrhea , disease , pathology , dermatology
Background Langerhans cell histiocytosis ( LCH ) is frequently known to involve multiple organ systems. However, gastrointestinal involvement by LCH is rare. Methods We describe a 68‐year‐old woman with a 3‐year history of intermittent diarrhea initially diagnosed as inflammatory bowel disease. She was subsequently found to have systemic LCH with involvement of the gastrointestinal tract, lungs, liver, and skin after skin biopsy was performed. A retrospective review of patients with cutaneous involvement of LCH seen at the M ayo C linic over the past 15 years was conducted. The presence of systemic disease as well as specific organ system involvement was reviewed. Results Twenty‐four patients with cutaneous LCH were identified. Besides our case, one other patient with both gastrointestinal and cutaneous involvement was identified. This patient died at six months of age. No other adult‐onset cases were identified. Conclusions Gastrointestinal involvement with LCH is rare, can be easily misdiagnosed, and likely portends a poor prognosis. In patients with ill‐defined systemic symptoms, cutaneous exam and biopsy have the potential to diagnose systemic disease.