Journal des syndromes génétiques et de la thérapie génique
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Keratoacanthoma in Xeroderma Pigmentosum: An Entity Beyond Guess

Radhika Varma, Priya Prathap

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder, characterized by photosensitivity, pigmentary changes, premature skin aging and increase in risk of developing malignant neoplasms of both skin and eyes. Keratoacanthoma (KA) is a rapidly growing benign skin tumor, occurring primarily in elderly light skinned individuals. Here, we report the occurrence of KA of the nose with an unusual morphology in a 12 year old boy with XP where, such an association of the disease in young age is exceedingly rare.