Xeroderma pigmentosum with limited involvement of the UV-exposed areas: a case report

A 21-year-old woman with skin type IV, who had developed photophobia and brown, spotty, hyperpigmented lesions on her face from early childhood, presented to our center for treatment of her facial lesions. Examination on admission revealed numerous, freckle-like, hyperpigmented macules and actinic keratoses over the central part of the face, with sparing of the forehead, chin, and peripheral area (Fig. 1). The area involved was approximated to be around 2% of the total body surface. The dorsal parts of the hands showed no lesions (Fig. 2), but guttate hypomelanotic lesions were apparent on both forearms. Figure 1. Limitation of xeroderma pigmentosum lesions to the center of the face Download figure to PowerPoint Figure 2. Hands are devoid of any lesions Download figure to PowerPoint Histologic examination of biopsies from four different facial lesions revealed them to be keratoacanthoma (1.5 × 2.5 cm ulcerative nodule on the right cheek), sclerosing basal cell epithelioma (nasal lesion), lentigo simplex, and hypertrophic actinic keratosis. Corneal clouding, conjunctival injection, loss of lashes, and atrophy of the lids were apparent on ophthalmologic examination. Other parts of the physical examination, including examination of the oral cavity, were nonsignificant. In addition, except for the presence of mild eczema in a sibling, the patient's family history regarding the presence of any similar problem and also any other important dermatologic or general disorder was negative.