Isolated cutaneous Langerhans cell histiocytosis in circumcised preputial skin
How to cite this article: Patra S, Trivedi P. Isolated cutaneous Langerhans cell histiocytosis in circumcised preputial skin. J Hematol Allied Sci 2021;1:84-5.
A 2-year-old child underwent circumcision for phimosis with clinical suspicion of balanitis xerotica obliterans. Histopathological examination of the preputial skin revealed diffuse dermal infiltration by sheets of atypical mononuclear cells [Figure 1a and b] having round to oval convoluted nuclei, nuclear grooves, inconspicuous nucleoli, and abundant eosinophilic cytoplasm [Figure 1c]. Few eosinophils were also evident. On immunohistochemistry, these cells expressed CD1a [Figure 1d] and S-100 diffusely. A diagnosis of cutaneous Langerhans cell histiocytosis (LCH) was rendered. The child was evaluated thoroughly but no other organ or system was involved. No residual lesion was detected by positron emission tomography scan. He had been kept in close follow-up for the past 6 months.
The differential diagnoses to consider are inflammatory conditions with florid histiocytic reaction and T-cell Non-Hodgkin Lymphoma. However, immunohistochemistry for CD1a, S-100, and T cell markers such as CD2, CD3, and CD5 usually solve the issue.
LCH, is a rare neoplastic condition of myeloid dendritic cells that express CD1a, langerin (CD207), S100, and cytoplasmic Birbeck granules. Isolated cutaneous LCH is extremely rare[1,2] and usually treated by surgical excision or corticosteroids or rarely multi-agent chemotherapy. Prognosis is good without other system involvement.
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