Roentgen Findings in Alkaptonuric Ochronosis

Two Cases of Alkaptonuria

“…The deposition of pigment takes place mainly in cartilage and tendons, and is followed by calcification in these sites; in joints this gives rise to arthritic changes. Pomeranz et al (1941) have described cutaneous pigmentation, and it rnay be that the skin pigmentation noted in Case 1 was the first manifestation of developing ochronosis. The sciera is often affected.…”

Vertebral changes in ochronosis.

“…(A) Calcium deposits, similar to those in the annulus fibrosus, have also been found in the pubic symphysis (Sit'aj, 1963), in the helix of the external ear (Mueller, Sorensen, Strandjord, and Kappas, 1965;Pomeranz, Friedman, and Tunick, 1941;Thompson, 1957), and in costal cartilage (Cooper and Moran, 1957). They have not been observed in diarthrodial articulations.…”

The skin in genetically-controlled metabolic disorders.

“…There is also a variable degree of fusion of vertebral bodies. The changes are so typical that the disease can be initially picked up by a radiologist (Pomeranz, Friedman, and Tunick, 1941). How far the pigmentation is due to the enzyme defect is debatable, as acquired cases have been seen after atabrine treatment (Ludwig, Toole, and Wood, 1963), and also after prolonged use of carbolic acid dressings for ulcers (Beddard, 1910;Brogren, 1952).…”