Gastrointestinal Tract Involvement in Langerhans Cell Histiocytosis: Case Report and Literature Review

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AbstractPulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. In this report, we present a 37-year-old male PLCH case that was negative for CD1a and S100 expression. Smoking cessation and use of prescribed Spiriva appeared to improve the patient’s symptoms. To the best of our knowledge, this is the first reported case of PLCH in which improved symptoms were seen with the use of Spiriva alone.The mechanism is not clear, but potentially has some relationship with dilating the airway, decreasing the mucous hypersecretion and promoting anti-inflammatory pathways. From this patient’s case, we may be able to find more cases to then find other first line therapies for PLCH patients.


Author(s):  
Shevachut Chavananon ◽  
Pornpun Sripornsawan ◽  
Thirachit Chotsampancharoen

An 8-month-old girl presented with chronic mucous bloody diarrhea for 3 months. She was diagnosed as infective gastroenteritis and did not improve after antibiotic treatment. Three months subsequently, she was diagnosed with allergic gastroenteritis after developing generalized skin rash and was treated with amino acid-based formula but the symptoms of diarrhea deteriorated. The results of a biopsy of the gastrointestinal tract and skin lesions confirmed the diagnosis of Langerhans cell histiocytosis. Although Langerhans cell histiocytosis is uncommon with gastrointestinal tract involvement, Langerhans cell histiocytosis should be listed in the differential diagnosis for patients who present with chronic mucous bloody diarrhea that is unresponsive to multimodality treatment.


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