© 2003 The International Society of Dermatology International Journal of Dermatology 2003, 42, 295 –296
295
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.
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.
Blackwell Science, Ltd Oxford, UK IJD International Journal of Dermatology 0011-9059 Blackwell Science, 2002 ?
Cameo
Xeroderma pigmentosum Mirshams-Shahshahani and Namazi Cameo
Xeroderma pigmentosum with limited involvement of the
UV-exposed areas: a case report
Mostafa Mirshams-Shahshahani,
1
MD, and Mohammad Reza Namazi, MD
From the
1
Dermatology Department, Tehran
University of Medical Sciences, Tehran, Iran,
2
Dermatology Department, Shiraz University
of Medical Sciences, Shiraz, Iran
Correspondence
Mohammad Reza Namazi, MD
PO Box 71955-687
Shiraz
Iran
E-mail: namazi_mr@yahoo.com
Discussion
The first case of xeroderma pigmentosum (XP) with limited
involvement of the sun-exposed surfaces is presented. This
exotic presentation is in clear contrast with the usual manifes-
tation of XP, which involves all sun-exposed sites.
XP represents a group of inherited disorders with an
estimated prevalence of 4 in 106.
1
Clinically, XP manifests as
actinic keratoses, and basal and squamous cell carcinomas, as
well as melanomas, which may develop during early childhood
in UV-exposed areas.
2
XP serves as the prototype of heritable disease with
increased sensitivity to cellular injury.
3
It is an autosomal
Figure 1 Limitation of xeroderma pigmentosum lesions to the
center of the face Figure 2 Hands are devoid of any lesions