| Summary: | Xeroderma pigmentosum (XP) is an autosomal recessive condition associated with a marked increase in the development of malignancies. We report a 10 year girl with freckles, lentigens and keratosis on the face and neck which had gradually developed from the age of two. History of marked photophobia was present. Few months prior she developed single firm, pigmented, non-tender, freely- mobile, sessile nodule over left ala of nose. Multiple frecklings with atrophic hypopigmented patches were distributed all over face and sparsely on neck. Histopathology examination (HPE) from growth over nasal ala revealed malignant tumour composed of spindloid to epithelioid cells arranged in sheets and nests in dermis suggestive of spitzoid melanoma. Fine needle aspiration cytology (FNAC) from left post-auricular lymph nodes revealed high cellularity comprising of many pleomorphic cells in clusters having enlarged irregular hyperchromatic nuclei. Wide local excision was done with nasolabial transposition of flap cover.
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