Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature

Abstract

Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple organ systems and particularly affects bone, skin, and lymph nodes. Perianal cutaneous Langerhans cell histiocytosis is a rare condition in adults, with 15 cases reported in the literature. Case We present the case of a 50-year-old hispanic man who presented with a 9-month history of pruritus ani and a personal history of diabetes insipidus. Punch biopsy confirmed a lesion of Langerhans cells origin but could not exclude Langerhans cell sarcoma because of limited sample size. An additional biopsy was planned as well as a positron emission tomography scan to determine the extent of disease spread. While the patient failed to follow up for repeat biopsy, the positron emission tomography scan was performed and was negative for metastatic disease. A stable perianal lesion of Langerhans cell histiocytosis with benign clinical features in a 50-year-old male despite lack of treatment is extremely rare and has not been described in the literature so far. Here, we review the presentation and workup of patients with Langerhans cell histiocytosis, review the relevant literature, and discuss treatment planning. Conclusion Perianal Langerhans cell histiocytosis is rare, and there should be a high index of suspicion with chronic or new perianal lesions, especially in a patient with a history of diabetes insipidus. It is also important to consider the patient’s full clinical course when it is not possible to reach a definitive pathological diagnosis before management.

Adult localized Langerhans cell histiocytosis: A case report

“…Depending on the number of systems involved in the patient, LCH is divided into single-system-LCH (SS-LCH) and multi-system-LCH (MS-LCH)[ 14 ]. SS-LCH is divided into SS-S (a single site involving the bone, skin, or lymph nodes) and SS-M (involving multiple parts of the bone or lymph nodes).…”

Langerhans cell histiocytosis in adults: literature review

“…Изменения были в виде множественных изъязвлений (n = 2) и оди ночных полипов с отграниченным или инфильтратив ным типом роста (n = 7); сведений по 1 больному не представлено [98]. У детей поражение ЖКТ отно сится к фактору плохого прогноза [99], в то время как у взрослых, по единичным наблюдениям, отрицатель ного прогностического значения поражение ЖКТ не имеет [98,100,101]. При появлении диспепсических жалоб необходимо учитывать поражение ЖКТ в каче стве одной из возможных причин.…”

Erythematous plaques on the skin folds

“…Adult Langerhans cell histiocytosis lesions are often located on the scalp, face, and external genitalia and most commonly affect the flexural and intertriginous area, where they can manifest as scaly, greasy rashes and small, erythematous papules 78…”