Multiple Elevated Red Spots in a Woman With Nausea, Altered Stools, and Weight Loss

LCH is a rare disease, seen in both children and adults. It affects various organs and has a widespread pattern of signs and symptoms. In adults, the estimated incidence is 1 or 2 cases per million.1Aricò M. Girschikofky M. Généreau T. et al.Langerhans cell histiocytosis in adults: report from the International Registry of the Histiocyte Society.Eur J Cancer. 2003; 39: 2341-2348Abstract Full Text Full Text PDF PubMed Scopus (384) Google Scholar The etiology of LCH still is unknown. The Langerhans cells are believed to originate from hematopoietic stem cells, which express CD1a and/or S100. Because the literature reports fewer then 20 adult cases,2Singhi A. Montgomery E. Gastrointestinal tract Langerhans cell histiocytosis: a clinicopathologic study of 12 patients.Am J Surg Pathol. 2011; 35: 305-310Crossref PubMed Scopus (68) Google Scholar it is assumed that gastrointestinal LCH is underestimated. Notably, most reported adult cases present as solitary polyps. In adults, skin lesions associated with extensive involvement of the small and large intestine is seldom seen.Our patient was treated with prednisolone and vinblastine according to the LCH-A1 multicentre study program,3Stockschlaeder M. Sucker C. Adult Langerhans cell histiocytosis.Eur J Haematol. 2006; 76 (363–336)Crossref PubMed Scopus (122) Google Scholar complicated by polyneuropathy. Because there was multi-organ involvement, the patient was maintained on therapy with daily 6-mercaptopurine and prednisolone for 5 days once every 3 weeks. Three months later, her symptoms improved, and the skin and intestinal lesions diminished. However, complete remission was not achieved.In conclusion, we report an interesting case of LCH in an adult with involvement of the skin and intestinal tract that was responsive to prednisolone, vinblastine, and 6-mercaptopurine. LCH is a rare disease, seen in both children and adults. It affects various organs and has a widespread pattern of signs and symptoms. In adults, the estimated incidence is 1 or 2 cases per million.1Aricò M. Girschikofky M. Généreau T. et al.Langerhans cell histiocytosis in adults: report from the International Registry of the Histiocyte Society.Eur J Cancer. 2003; 39: 2341-2348Abstract Full Text Full Text PDF PubMed Scopus (384) Google Scholar The etiology of LCH still is unknown. The Langerhans cells are believed to originate from hematopoietic stem cells, which express CD1a and/or S100. Because the literature reports fewer then 20 adult cases,2Singhi A. Montgomery E. Gastrointestinal tract Langerhans cell histiocytosis: a clinicopathologic study of 12 patients.Am J Surg Pathol. 2011; 35: 305-310Crossref PubMed Scopus (68) Google Scholar it is assumed that gastrointestinal LCH is underestimated. Notably, most reported adult cases present as solitary polyps. In adults, skin lesions associated with extensive involvement of the small and large intestine is seldom seen. Our patient was treated with prednisolone and vinblastine according to the LCH-A1 multicentre study program,3Stockschlaeder M. Sucker C. Adult Langerhans cell histiocytosis.Eur J Haematol. 2006; 76 (363–336)Crossref PubMed Scopus (122) Google Scholar complicated by polyneuropathy. Because there was multi-organ involvement, the patient was maintained on therapy with daily 6-mercaptopurine and prednisolone for 5 days once every 3 weeks. Three months later, her symptoms improved, and the skin and intestinal lesions diminished. However, complete remission was not achieved. In conclusion, we report an interesting case of LCH in an adult with involvement of the skin and intestinal tract that was responsive to prednisolone, vinblastine, and 6-mercaptopurine.