Adult cutaneous Langerhans’ cell histiocytosis: a rare presentation, successful treatment with thalidomide

P.L. Chandravathi, Praneet Awake, Anil Fonseca

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.


Keywords


Langerhans’cell histiocytosis, cutaneous, adult, thalidomide

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References


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.

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.

Sander CS, Kaatz M, Elsner P. Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide. Dermatology. 2004;208:149-52.

Götz G, Fichter J. Langerhans' cell histiocytosis in 58 adults. Eur J Med Res. 2004;9:510-4.

Walia SS, Gayer K. An unusual presentation of refractory cutaneous Langerhans cell histiocytosis in an adult. J Drugs Dermatol. 2006;5:174-7.

Kolde G, Schulze P, Sterry W. Mixed response to thalidomide therapy in adults: two cases of multisystem Langerhans' cell histiocytosis. Acta Derm Venereol. 2002;82:384-6.

Conias S, Strutton G, Stephenson G. Adult cutaneous Langerhans-cell histiocytosis. Australas J Dermatol. 1998;39:106-8.


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