Langerhans cell histiocytosis (LCH) mainly affects children and rarely occurs in adults [1]. Mostly, LCH presents as single system disease (SS) affecting only one organ system; in multisystem disease (MS), bone, skin, mucosae, lung, liver, spleen, bone marrow, lymph nodes, hypothalamus and the pituitary gland can be involved [1-3]. We report two patients with perianal skin ulcerations as the initial symptom of LCH. Both were successfully treated with intravenous vinblastine and oral prednisone, according [...]