We searched PubMed for articles published between 1974 and 2017 using the search terms "alkaptonuria", "
ochronosis", "ochronotic arthropathy", "ochronotic tendinopathy", and "alkaptonuria and osteoporosis." We also checked the references in the retrieved articles.
Alkaptonuria,
ochronosis, and ochronotic arthropathy.
"Exogenous
Ochronosis: An Epidemiological Study." British Journal of Dermatology.
Ochronosis and arthritis typically only occur after the third decade, and patients are often unaware they have the condition until adulthood.
Keywords: Alkaptonuria; lumbar disc hernia;
ochronosis.
The histologic features of the surgical specimen were compatible with
ochronosis and osteonecrosis [Figure 1]d.
Recent advances in the understanding of
ochronosis pathophysiology opened new therapeutic perspectives and suggest possible benefits of antioxidants [68].
HGA-oxidized derivative benzoquinone acetic acid (BQA) forms melanin-based polymers, deposited in the connective tissue of various organs, causing a pigmentation known as "
ochronosis," leading to dramatic tissue degeneration.
High doses of arbutin induce chronic poisoning of the skin, resulting in
ochronosis [24].
Homogentisate polymers accumulate and cause urine darkening (called alkaptonuria), brown black pigmentation of connective tissue (called
ochronosis), articular cartilage pathology, osteoporosis and pathomorphologic changes in internal organs.
Studies have shown that long term hydroquinone use can cause exogenous
ochronosis, which is when your skin turns a bluish and black colour Hydroquinone not only inhibits melanin production to help lighten skin, but long term use can actually damage your pigment cells.
Alkaptonuria is a rare metabolic disorder in the phenylalanine and tyrosine catabolic pathway which is characterized by the excessive excretion of homogentisic acid in the urine,
ochronosis, and debilitating arthritis of the spine and large joints.