Ann Dermatol. 1996 Oct;8(4):278-281. English.
Published online Nov 28, 2016.
Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology
Case Report

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

Ki Soon Nam, Jung Youl Lee, Kyu Uang Whang and Young Keun Kim

    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.

    Keywords
    Hunter's syndrome


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