Adult Cutaneous Langerhans' Cell Histiocytosis: a Rare Presentation, Successful Treatment with Thalidomide. | [Journal of Pakistan Association of Dermatologists • 2016]

Author(s):

1. PL Chandravathi: Department of Dermatology, Venereology and Leprosy, Care Institute of Medical Sciences, Hyderabad, Telangana, India

2. Praneet Awake: Department of Dermatology, Venereology and Leprosy, Care Institute of Medical Sciences, Hyderabad, Telangana, India

3. Anil Fonseca: Department of Pathology, Care Institute of Medical Sciences, Hyderabad, Telangana, India

Abstract:

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.

Page(s): 143-146

DOI: DOI not available

Published: Journal: Journal of Pakistan Association of Dermatologists, Volume: 26, Issue: 2, Year: 2016

Keywords:

Keywords are not available for this article.

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