Xeroderma pigmentosa--a disfiguring disease.

ABSTRACT

Xeroderma pigmentosa is a rare inherited autosomal recessive disease with the inability to repair DNA damage caused by UV light. Recognized in the late 1800 by Maritz Kaposi it has been reported world wide and in all races with an over prevalence of 1-4 per million population. Kunwar et al. Those affected are extremely sensitive to the UV portion of the light and have a 2000-fold increased risk of skin cancer in the sun exposed skin. Basal cell carcinoma is the most commonly associated carcinoma followed by Squamous cell carcinoma and Melanoma. The pigmentation on the face and the rest of the body can be horribly disfiguring. The recurring cancer occurring on the face and repeated surgical treatment for the ulcerations have important social and psychological implications not encountered with other cancers. We report two cases of BCC and melanoma. The first case is of BCC of the face in a teenaged girl coexisting with xeroderma pigmentosa. The second case presented with melanoma of the scalp in a 10 year old female child. The details of these cases are presented and the management.