A 31-year old man with corneal clouding and stiff joints, who may represent a newly recognized genetic form of systemic mucopolysaccharidosis type VI (MaroteauxLamy syndrome), was studied by histochemistry and electron microscopy. Connective tissue cells of cornea, conjunctiva, and skin had vacuoles containing acid mucopolysaccharide. This change was most marked in the cornea. Plasma cells with Russell bodies frequently occurred in the conjunctival stroma.
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