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May 1977

A Cutaneous Marker in the Hunter Syndrome: A Report of Four Cases

Author Affiliations

From the Division of Dermatology (Drs Prystowsky and Herndon), Medical Genetics of the Department of Internal Medicine (Dr Harrod), and Department of Pathology (Dr Freeman), University of Texas Southwestern Medical School, Dallas; and the Departments of Medicine and Ophthalmology (Dr Maumenee), Johns Hopkins University School of Medicine and Johns Hopkins Hospital, Baltimore. Dr Prystowsky is now with the Letterman Army Institute of Research, San Francisco.

Arch Dermatol. 1977;113(5):602-605. doi:10.1001/archderm.1977.01640050062007

• A report of four patients and review of the literature suggest that a cutaneous marker exists for the Hunter syndrome. All previously described patients with this eruption have been males with clear corneas. The distinctive lesions consist of firm ivory-white papules and nodules that may coalesce to form ridges or a reticular pattern. The papules are usually seen in symmetrical areas between the angles of the scapulas and posterior axillary lines, the pectoral regions, the nape of the neck and/or on the lateral aspects of the upper arms and thighs. These lesions are seen in both allelic forms and cannot be used to separate a benign from a rapid course.

(Arch Dermatol 113:602-605, 1977)

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