Last Updated on August 7th, 2019
SAPHO syndrome is a condition that affects up to 1% of the population of the United States—and up to 4% of the population in Alaska and Denmark—that most of us have never heard of. Sometimes merged with a condition called hidradenitis suppurativa1, SAPHO syndrome is characterized by a combination of synovitis (inflammation of the linings of the joints), acne, pustulosis (formation of cysts without infection), hyperostosis (knotty growths on the bones), and osteitis (bone irritation). Its symptoms show up most commonly in humid climates.
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SAPHO syndrome can appear at any time between childhood and middle age and usually follows a relapsing and remitting course. Symptoms get worse and they get better and they get worse again, with or without treatment. Sometimes medications that actually help the condition don’t seem to work and medications that don’t help the condition appear as if they do.
At the level of the skin, one of SAPHO’s most recognizable symptoms2 is a kind of acne known as palmoplantar pustulosis, which breaks out on the palms of the hands and the soles of the feet. There can also be acne conglobata, a kind of acne that causes pimples to burrow tunnels to each other and merge into large sores, or hidranenitis suppurativa, which is a form of acne caused by the skin growing over pores rather than infection in pores, and acne fulminans, in which the skin is free of acne bacteria but they are found growing in joints.
SAPHO syndrome can cause unremitting bone pain that is worse at night. It can cause joints and bones to become tender, so that just touching the skin causes pain. The pain caused by SAPHO syndrome can last for years, and cause disruption of the entire family of the person who suffers from the condition.
Some doctors believe that SAPHO syndrome is caused by the same autoimmune processes that cause ulcerative colitis, a severe inflammatory disease of the bowel. There is a specific blood marker for SAPHO syndrome3 called HLA-B27, which appears in the bloodstream in people who get SAPHO syndrome, ulcerative colitis, ankylosing spondylitis, and another condition that causes problems in both the skin and joints, psoriatic arthritis.
People of certain ethnic groups are far more likely to carry this bloodstream marker than others. Surveys find that members of the native Alaskan Yupik people4, Tarahumara Indians in Mexico and their Pima cousins in Arizona, Danes, Finns, Belgians, and Irish people are especially likely to have the bloodstream marker. Everyone who carries the marker in their bloodstream does not get a disease, but symptoms of the disease plus the marker are used to make the diagnosis.
It is very rare for SAPHO syndrome to appear before the age of 10 or after the age of 50. It is much more common in women than in men, by a ratio of somewhere between two and five to one. Children and teens who get SAPHO syndrome tend to have acne breakout on their hands, feet, and under their arms. Women who get SAPHO syndrome tend to have acne breakout on their hands, feet, and genitals. Men who get SAPHO syndrome tend to have acne breakout on their hands, feet, and perianal region.
The first priority in treating SAPHO syndrome is pain relief5. In most of the world, this means taking massive amounts of non-steroidal anti-inflammatory agents (NSAIDs), such as aspirin, Ibuprofen, and Tylenol. Managing the pain of SAPHO syndrome with NSAIDs is tricky, because the quantities of pain relievers required usually cause digestive upset. The kinds of medications that are used with other kinds of bone and joint pain, such as Vicodin and Oxycontin, usually don’t work especially well for the pain of SAPHO syndrome.
Doctors sometimes try to treat the destruction of bones and joints caused by SAPHO syndrome with the same drugs used for rheumatoid arthritis, such as sulfasalazine (Azulfidine), methotrexate (Rheumatrex, Trexall), and infliximab (Remicade). The best results usually come from the use or infliximab, but the cost of treatment can be quite high without health insurance.
Some people who get SAPHO are so bothered by their bone pain that they don’t worry about acne, but as patients recover from pain and immobility they then want to improve their appearance. For the pustules that appear on the hands and palms, the best self-care is remembering to wash both hands and feet at least once a day and keeping the palms of the feet dry. Wearing socks that wick moisture out and away from the skin helps. Doctors may offer radiofrequency treatments for pustules on the palms.
For the acne that breaks out below the belt and under the arms, it is especially helpful to wear loose, dry clothing, and to avoid the accumulation of perspiration on the skin. This allows the skin to exfoliate itself so the pimples eventually open up—you don’t want to use an acid peel or an acid exfoliant, or an exfoliant cloth, on delicate skin. When acne lesions become especially swollen and painful, the doctor can inject them with a corticosteroid6 such as prednisolone (Prelone) or triamcinolone (Amcort, Aristospan Intra-Articular). Repeated use of injected steroids at the same spot can cause the skin to thin and loose coloration.
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