A Case of Hunter's Syndrome With Ivory-colored Papules.
10.5021/ad.1996.8.4.278
- Author:
Ki Soon NAM
;
Jung Youl LEE
;
Kyu Uang WHANG
;
Young Keun KIM
- Publication Type:Case Report
- Keywords:
Hunter's syndrome
- MeSH:
Animals;
Arm;
Child;
Child, Preschool;
Contracture;
Cornea;
Hand;
Hoof and Claw;
Humans;
Hypertrichosis;
Joints;
Male;
Mucopolysaccharidosis II*;
Neck;
Scapula;
Siblings
- From:Annals of Dermatology
1996;8(4):278-281
- CountryRepublic of Korea
- Language:English
-
Abstract:
We report a case of Hunter' s syndrome with characteristic nodules on the upper back. The patient was a 7-year-old Korean boy who presented with ivory-colored papules and nodules on both sides of the scapula, pectoral regions and lateral aspects of the upper arms. These lesione are regarded as pathognomonic cutaneous markers for Hunter s syndrome. He also presented with truncal hypertrichosis, retarded growth, short neck, round face, claw like contractures of hands, multiple joint contractures, and a clear cornea. Severely elevated glycosaminoglycan levels were present in the patient s urine samples. The patient s 5-year-old brother had similar clinical features.
