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Xeroderma pigmentosum  

Xeroderma pigmentosum (XP) is a neurocutaneous disorder with an autosomal recessive mode of inheritance. The skin including the lips is affected, showing epithelial atrophy and hyperpigmentation. These patients are extremely sensitive to light and show an increased predisposition to UV-associated malignancies of the skin. Carcinoma of the tongue has also been reported . The diagnosis of Xeroderma pigmentosum is established by biopsy. Use of sunscreen and avoidance of sun exposure are recommended. Oral retinoids have shown some benefit in the prevention of neoplasms in patients with XP.

Image	Caption
	Figure 1: Carcinoma of the tip of the tongue in this 19–year-old boy with xeroderma pigmentosum.
	Figure 2: The same 19–year-old patient with xeroderma pigmentosum. Note the hyperpigmented skin lesions.
	Figure 3: The same 19–year-old patient with squamous cell carcinoma of the skin (arrow) with xeroderma pigmentosum.