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A Pathological Basis for the Cutaneous Papules of Mucopolysaccharidosis II (The Hunter Syndrome)
Author(s) -
FREEMAN ROBERT G.
Publication year - 1977
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.1977.tb00923.x
Subject(s) - pathological , mucopolysaccharidosis , pathology , medicine , dermatopathology , dermatology , mucopolysaccharidosis i , disease , enzyme replacement therapy
Cutaneous papules of two patients with the Hunter syndrome (mucopolysaccharidosis II) were compared with their normal appearing skin by light and electron microscopic 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.