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April 2000

Extensive Bluish Gray Skin Pigmentation and Severe Arthropathy

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Arch Dermatol. 2000;136(4):547-552. doi:

A 65-year-old man presented with a 30-year history of slowly developing extensive bluish gray skin pigmentation. For about 2 decades, he had increasing pain in the lumbar region, hips, and knees. His mother, who had similar clinical signs, remembered that the patient's diapers had shown discoloration when he was a baby.

Physical examination revealed prominent bluish gray pigmentation of the face (Figure 1), hands, and cartilage of the ears and nose. Pigmentation of extensor tendons was prominent and could be seen through the skin of the hands (Figure 2). There were grayish axillary skin pigmentation, similar pigmentary changes of the nailbed, and black cerumen. On the right sclera, triangular brown pigmentation appeared laterally. A biopsy specimen was obtained from a pigmented area (Figure 3).

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