title: A cutaneous marker in the Hunter syndrome a report of four cases.
abstract: 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.
mesh_terms: D001132:Arm; D002648:Child; D006228:Hand Deformities, Congenital; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D016532:Mucopolysaccharidosis II / Q000235:genetics / Q000473:pathology*; D012540:Scapula; D012877:Skin Manifestations; D013848:Thigh; D013909:Thorax