Conflicts of interest: None.
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
Version of Record online: 28 APR 2013
© 2013 The International Society of Dermatology
International Journal of Dermatology
Volume 53, Issue 3, pages 305–311, March 2014
How to Cite
Podjasek, J. O., Loftus, C. G., Smyrk, T. C. and Wieland, C. N. (2014), 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. International Journal of Dermatology, 53: 305–311. doi: 10.1111/j.1365-4632.2012.05716.x
- Issue online: 20 FEB 2014
- Version of Record online: 28 APR 2013
Langerhans cell histiocytosis (LCH) is frequently known to involve multiple organ systems. However, gastrointestinal involvement by LCH is rare.
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.
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.
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.