Adult-onset systemic Langerhans cell histiocytosis mimicking inflammatory bowel disease: the value of skin biopsy and review of cases of Langerhans cell histiocytosis with cutaneous involvement seen at the Mayo Clinic.

Autor: Podjasek JO; Department of Dermatology, Mayo Clinic, Rochester, MN, USA., Loftus CG, Smyrk TC, Wieland CN
Jazyk: angličtina
Zdroj: International journal of dermatology [Int J Dermatol] 2014 Mar; Vol. 53 (3), pp. 305-11. Date of Electronic Publication: 2013 Apr 28.
DOI: 10.1111/j.1365-4632.2012.05716.x
Abstrakt: 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 Mayo Clinic 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.
(© 2013 The International Society of Dermatology.)
Databáze: MEDLINE
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