Abstract
A report of 4 patients and review of the literature suggest that a cutaneous marker exists for the Hunter syndrome. All previously described patients with this eruption were 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.

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