A Pathological Basis for the Cutaneous Papules of Mucopolysaccharidosis II (The Hunter Syndrome)

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Abstract
Cutaneous papules of 2 patients with the Hunter syndrome (mucopolysaccharidosis II) were compared with their normal appearing skin by light microscopic and EM techniques. While most fibrocytes of normal and papular skin contained metachromatic cytoplasmic material and characteristic vacuoles, only the papules had extracellular accumulation of metachromatic material and evidence of coalescence and rupture of vacuoles. Alcohol fixation provides better preservation of the mucosubstances.