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VOLUME 64 , ISSUE 4 ( October-December, 2022 ) > List of Articles

CASE REPORT

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Pankaj Gupta, Abhijeet Singh, Indermohan Chugh, Pratik Patil, Promila Pankaj

Keywords : Adult, Langerhans cell histiocytosis, Perianal, Vulva

Citation Information : Gupta P, Singh A, Chugh I, Patil P, Pankaj P. Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement. Indian J Chest Dis Allied Sci 2022; 64 (4):277-287.

DOI: 10.5005/jp-journals-11007-0036

License: CC BY-NC 4.0

Published Online: 05-01-2023

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

Langerhans cell histiocytosis (LCH) also known as histiocytosis X, is a rare systemic disorder arising from the clonal proliferation of myeloid dendritic cells (histiocytes) with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. Clinical presentation usually depends on the site of involvement. The organs commonly affected in adults by order of decreasing frequency include lungs, bone, skin, pituitary glands, lymph nodes, and the liver. Vulval and perianal involvement is extremely rare in adults. We describe the case of a 31-year-old non-smoker adult female with multisystemic LCH involving the vulva, perianal region, and lung. Probable involvement of other sites with LCH included mandibular bone, pituitary gland, skin, lymph nodes, liver, thyroid, and colon. She is undergoing systemic chemotherapy and has completed two cycles of cytarabine and steroids without any complications. Treatment is not standardized due to the very less incidence of the disease and inadequate knowledge regarding its pathophysiology. Langerhans cell histiocytosis remains a major concern for treating physicians because of its rarity with many faces and requires careful consideration for management.


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