Generalized Acanthosis Nigricans in Early Childhood Dusan S. Skiljevic, M.D.,* Milos M. Nikolic, M.D., Ph.D.,* Anica Jakovljevic, M.D.,² and Danijela D. Dobrosavljevic, M.D., M.S.* *Institute of Dermatology, University Clinical Center, Belgrade, Yugoslavia, and ²Department of Pediatrics, Novi Sad, Yugoslavia Abstract: Acanthosis nigricans (AN) is a disorder often accompanied by internal malignancy in adult patients, but in children it is usually be- nign. Generalized forms of AN, especially in childhood, are extremely rare. We report a 5-year-old boy with a 3-year history of AN who had generalized roughness and hyperpigmentation, numerous skin wrinkles, disseminated skin tags, tripe palms, and intensive pruritus. No clinical evidence of internal disorders, notably of an endocrinologic or neoplastic nature, have been detected thus far. In 1889 Pollitzer and Janovsky independently de- scribed a disease which Unna named acanthosis nigri- cans (AN). In 1893 Darier made a connection between AN and internal malignancy (1). AN is characterized by roughness and thickening of the skin, with hyperpigmentation and small papilloma- tous elevations—acrochordons predominantly in the ax- illae, anogenital region, groin, submammary region, um- bilicus, and on the back and sides of the neck (2–4). The disorder can be inherited or it can be associated with an endocrine abnormality, obesity, use of certain drugs, and malignancy (2–4). The onset of AN in adulthood may be a sign of internal malignancy. On the other hand, the vast majority of cases of AN in childhood are of a benign hereditary form or a benign form related to insulin re- sistance (2,3). Generalized forms of AN are extremely rare, espe- cially in childhood. We describe a 5-year-old boy with a 3-year history of generalized AN and tripe palms, with no signs of internal disorders. CASE REPORT The patient was a boy, born in 1995, the first and only child of healthy Caucasian parents of Hungarian ances- try. He was born 10 days past term; the delivery was induced and finished by vacuum extraction. His early physical and mental development were nor- mal. History revealed that the first signs of disease com- menced at the age of 2 years, with onset of pruritus, dry skin, the appearance of wrinkles in the axillae, and white “warts” on the face and trunk. On physical examination, the boy was in good general health, although scratching himself intensely. His body mass was 18 kg, height 1.06 m, and body mass index 15.93, normal for his age. Skin examination showed generalized hypertrophy and hyperpigmentation. The entire integument had a vel- vety texture. Changes were most pronounced on the face, neck, axillae, and groins (Fig. 1). Numerous small, el- evated and pedunculated white tumors, 1–4 mm in di- ameter (acrochordons), were also present, especially on Address correspondence to Milos M. Nikolic, M.D., Institute of Dermatology, University Clinical Center, Pasterova 2, 11000 Belgrade, Yugoslavia, or e-mail: mmnikolic@ptt.yu. Pediatric Dermatology Vol. 18 No. 3 213–216, 2001 213