A cutaneous marker in the Hunter syndrome a report of four cases

Arch Dermatol. 1977 May;113(5):602-5.


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.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Arm
  • Child
  • Hand Deformities, Congenital
  • Humans
  • Intellectual Disability / etiology
  • Male
  • Mucopolysaccharidosis II* / genetics
  • Mucopolysaccharidosis II* / pathology*
  • Scapula
  • Skin Manifestations*
  • Thigh
  • Thorax