Ann Dermatol.  1996 Oct;8(4):278-281. 10.5021/ad.1996.8.4.278.

A Case of Hunter's Syndrome With Ivory-colored Papules

• Nam KS
• Lee JY
• Whang KU
• Kim YK

• KMID: 2360451
• DOI: http://doi.org/10.5021/ad.1996.8.4.278

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.

Keyword

Hunter's syndrome

MeSH Terms

Animals
Arm
Child
Child, Preschool
Contracture
Cornea
Hand
Hoof and Claw
Humans
Hypertrichosis
Joints
Male
Mucopolysaccharidosis II*
Neck
Scapula
Siblings