Adult cutaneous Langerhans’ cell histiocytosis: a rare presentation, successful treatment with thalidomide
DOI:
https://doi.org/10.66344/jpad.v26i2.57Keywords:
cutaneous, adult, thalidomide, Langerhans’ cell histiocytosisAbstract
Langerhans’ cell histiocytosis (LCH) is a rare clonal disorder of proliferating histiocytic cells expressing phenotypic markers of the epidermal Langerhans’ cells. LCH generally affects children. Adult LCH with single system disease limited to skin is uncommon and difficult to diagnose. The management of LCH is difficult as these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. We report a case of 48 year female, diabetic and hypertensive presented as single system LCH limited to skin with history of recurrent painful erythematous ulcerated lesions in right axilla and both groins since 4 years with and limiting mobility of limbs. She was successfully treated with thalidomide. Thalidomide monotherapy represents an effective, safe and well-tolerated treatment option that should be considered as first-line therapy for single system LCH limited to skin which is rare and difficult to treat.
References
1. Pan Z, Sharma S, Sharma P. Primary langerhans cell histiocytosis of the vulva: report of a case and brief review of the literature. Indian J Pathol Microbiol. 2009;52:65-8.
2. Nezelof C, Basset F. An hypothesis Langerhans cell histiocytosis: the failure of the immune system to switch from an innate to an adaptive mode. Pediatr Blood Cancer. 2004;42:398-400.
3. Sander CS, Kaatz M, Elsner P. Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide. Dermatology. 2004;208:149-52.
4. Götz G, Fichter J. Langerhans' cell histiocytosis in 58 adults. Eur J Med Res. 2004;9:510-4.
5. Walia SS, Gayer K. An unusual presentation of refractory cutaneous Langerhans cell histiocytosis in an adult. J Drugs Dermatol. 2006;5:174-7.
6. Kolde G, Schulze P, Sterry W. Mixed re-sponse to thalidomide therapy in adults: two cases of multisystem Langerhans' cell histiocytosis. Acta Derm Venereol. 2002;82:384-6.
7. Conias S, Strutton G, Stephenson G. Adult cutaneous Langerhans-cell histiocytosis. Australas J Dermatol. 1998;39:106-8.
Downloads
Published
Issue
Section
License
Copyright (c) 2016 P.L. Chandravathi, Praneet Awake, Anil Fonseca

This work is licensed under a Creative Commons Attribution 4.0 International License.
Submission declaration
Authors retain the copyright to their work and grant the 'Journal of Pakistan Association of Dermatologists (JPAD)' the right of first publication under a Creative Commons Attribution 4.0 International (CC BY 4.0) license. This license allows others to share, adapt, and reuse the work for any purpose, including commercial use, as long as appropriate credit is given to the original authors and the journal.
By submitting a manuscript, authors confirm that the work has not been published previously (except as an abstract, lecture, or academic thesis), is not under review elsewhere, and has been approved by all authors and relevant authorities. Once accepted, the article will be openly accessible under the CC BY 4.0 license, ensuring wide dissemination and reuse with proper attribution.






