Langerhans cell histiocytosis of skin: a clinicopathologic analysis of five cases.

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2006-05-13
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BACKGROUND AND AIMS: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. METHODS: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. RESULTS: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells) with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. CONCLUSION: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.
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Punia RS, Bagai M, Mohan H, Thami GP. Langerhans cell histiocytosis of skin: a clinicopathologic analysis of five cases. Indian Journal of Dermatology, Venereology and Leprology. 2006 May-Jun; 72(3): 211-4