Conjunctiva and skin from nine patients representing each of the six systemic mucopolysaccharidoses were examined by histochemistry and electron microscopy. Connective tissue cells consistently exhibited abnormal vacuolation by single-membrane-limited cytoplasmic bodies. These vacuoles, which contained predominantly fibrillogranular and occasionally membranous lamellar inclusions, are thought to represent lysosomes engorged with excessive accumulation of acid mucopolysaccharide and glycolipid, respectively. Among all specimens, the extent of vacuolation was more variable in conjunctiva than in skin. The extent of cellular abnormality in the conjunctiva did not consistently parallel to degree of corneal opacification.
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