A Case of Xeroderma Pigmentosum / 대한피부과학회지

ABSTRACT

Xeroderma pigmentosum is characterized by sunlight sensitivity, autosomal recessive inheritance, and multiple cutaneous malignant melanoma, angiosarcoma and fibrosarcoma. Some patient also demonstrate neurological manifestations, ie, mental deficiency, microcephaly, ataxia, choreoathetosis, and deafness. A 22 year old Korean soldier was seen at the Capital Armed Forces General Hospital in May of 1974 because of pigmentation and ulceration on the exposed area. Since the age of 3, the patient has suffered from freckles on exposed area and the lesions were gradually increased in number. About 3 years ago, he observed nodule on the left side of face, which underwent ulceration eventually. There were no history of unconsciousness, convulsion or other neurological symptoms. The histopathological picture in the margin of ulcerated lesion revealed hyperkeratosis and acanthosis in epidermis, and masses of various shaped and sized composed of basalioma cells in dermis. The peripheral cell layer of the tumor masses showed pallisade arrangement of the nuclei.