Clinical manifestation

Dark urine in diapers usual first sign of disease; gray-black scleral pigmentation in configuration of small, dark rings; ear cartilage discoloration with a grayish-blue hue, followed by structural changes with stiffness, contour irregularities, and calcification; discoloration on nasal tip, costochon-dral junctions, extensor tendons of the hands, cheeks, fingernails, and buccal mucosa; ochronotic arthropathy; ochrono-sis-like pigmentation as idiosyncratic reaction to application of hydroquinone or phenol

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