Alkaptonuria with ochronotic arthritis.
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The use of hydroquinone as a cosmetic skin-bleaching agent has been forbidden since January 2001. It is now available only on prescription. The ban has been introduced because of medium-term effects such as white patches on the skin, particularly on the face (leukoderma with confetti-like depigmentation), and subcutaneous dark collections of pigment (exogenous ochonosis). Long-term effects are a possibility; cancer being the most likely. Renal adenomas and leukaemia occurred in animal experiments indicating the nephrotoxicity and carcinogenic properties of the substance. It is now known how hydroquinone and its metabolites can cause damage to DNA and inhibit apoptosis of mutated cells. The carcinogenic action of benzene is difficult to attribute to its hydroquinone metabolite. Daily use of hydroquinone causes it to accumulate in the body as absorption into the skin is faster than excretion in the urine. The use of hydroquinone as a skin-bleaching agent is accordingly unsafe and should be completely banned. Alternatives such as azaleic acid and thioctic acid (alpha-lipoic acid) are available.
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Alkaptonuria is a rare, autosomal-recessive, inborn, metabolic disease that causes ochronotic arthropathy leading to disabling arthritis. Few reports of arthroplasty in patients with this disease have been published, and none documents four total joint replacements in the same patient. This case report describes the history and findings in a 70-year-old man who underwent bilateral hip and knee total joint arthroplasties with significant improvement in pain and exercise tolerance. The metabolic and clinical aspects of alkaptonuria are also discussed.
After description of etiopathogenetic, anatomopathological and clinical aspects of the disease, the authors relate about a case of ochronotic arthropathy, lining on its typical radiological patterns. Scintigraphic study with 99mTc-pertechnetate and with 99mTc-pyrophosphate, excluding any phlogistic component, agree with dysmetabolic and degenerative nature of this arthropathy.
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