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A Cutaneous Marker in the Hunter Syndrome

1977; American Medical Association; Volume: 113; Issue: 5 Linguagem: Inglês

10.1001/archderm.1977.01640050062007

1538-3652

Stephen D. Prystowsky, Irene H. Maumenee, Robert G. Freeman, James H. Herndon, Mary Jo Harrod,

Cutaneous lymphoproliferative disorders research

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.