Langerhans cell histiocytosis

Blesa, Joan Manel Gasent; Candel, Vicente Alberola; Vercet, Carlos Solano; Canales, Juan Laforga; Semler, Christof; Antolí, M. Rosa Pérez; Rodriguez-Galindo, Carlos

doi:10.1590/s1516-31801998000100006

Cited by 1 publication

(1 citation statement)

References 3 publications

(2 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…2016 [ 8 ] 45 F Perianal wound (1 year) 3-cm ulcerovegetant purulent mass Pressure sensation Topical steroids 0 Femur and tibial lesions Methotrexate (MTX) Skin lesion healed with MTX Yes (1 M) Chauffaille et al. 1998 [ 9 ] 31 F Polydipsia (3 years), vulvar ulcers (1 year) Granulomatous perianal and vulvar ulceration – – 0 Femurs, skull, shoulder, forearm lesions, liver, oral lesions, DI, hypothalamic tumor on brain MRI – – – Mittal et al. 2009 [ 10 ] 45 M Perianal ulcerations (4 M) – Painful perianal ulcerations Nitrogen mustard, steroids, pentostatin.…”

Section: Discussionmentioning

confidence: 99%

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

2021

View full text Add to dashboard Cite

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.

show abstract