Perianal Langerhans cell histiocytosis

Abstract

Dear Editor, Langerhans cell histiocytosis (LCH) rarely occurs in adults and is usually a multi-focal systemic disease. We present the case of a 70-year-old male with sole perianal histiocytosis and discussion of current literature and management options. LCH is a rare disease characterised by the presence of Langerhans cell histiocytes in a variety of tissues and by a spectrum of clinical manifestations. Typically, LCH is a disease of childhood and is comparatively rarely reported in adults. LCH involving the GI tract is rare. A 70-year-old gentleman was referred to our colorectal clinic with a 3week history of fresh rectal bleeding associated with a 4-month history of perianal pain and mucous discharge. There was no associated change in bowel habit, weight loss or family history of colorectal disease. His past medical history included prostate cancer diagnosed in 2003. Anorectal examination revealed a 2-cm ulcerated lesion in the anal canal with perianal extension circumferentially. Further examination was prevented in the clinic setting by pain. Examination under anaesthetic confirmed that the lesion arose from the outside of the anal canal, with ulceration of the perianal skin, the rectum was normal. Biopsies were taken from different areas of the lesion for histological examination. Histological examination of the biopsies revealed squamous epithelial lined tissue with ulceration, granulation tissue and inflammation. Sheets of mononuclear cells with medium-sized normochromic, vesicular and indented nuclei along with abundant pale cytoplasm were present. A number of eosinophils were also present. Michaelis–Guttman bodies (characteristic findings in malakoplakia) were not identified. Mitotic figures were infrequent. This mononuclear infiltrate was positive for immunohistochemical markers for Langerhans cells and histiocytes including CD1a, S100 and CD68. The gentlemen went on to have topical treatment with potassium permanganate and steroids. Later, the patient developed a bone lesion in his left tibia which was biopsied and histological examination revealed histiocytic infiltration of bone with associated osteosclerosis. This was not associated with eosinophilic infiltrate and CD1a stain was negative. The diagnosis of Erdheim–Chester disease was made on this bone biopsy. Although Erdheim–Chester disease has been previously been classified as a type of LCH, it is now thought to be a distinct entity and no cases involving perianal lesions are described. The first published description of Langerhans histiocytosis was in 1865 in a 4 1/2-year-old with impetigo type M. Field . N. Inston . N. Cruickshank Department of Surgery, Sandwell and West Birmingham Hospital, Lyndon, West Bromwich, B71 4HJ, UK