Synovitis acne pustulosis hyperostosis osteitis
Overview
Syndrome consisting of synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis (SAPHO). The most common site of the disease is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions, hyperostosis, and arthritis of the adjacent joints. The association of sterile inflammatory bone lesions and neutrophilic skin eruptions is indicative of this syndrome. Source - Diseases Database
Symptoms
* Acne conglobata * Acne fulminans * Hydradenitis suppurativa * Exfoliating pustules on palms * Exfoliating pustules on soles * Sterno-costo-clavicular hyperostosis * Chronic osteomyelitis * Spondyloarthropathy * Skin lesions * Inflammation of joint lining * Sacroilitis syndesmophytes * Anterior chest lesions * Excessive localized bone growth - usually where bones and tendons attach * Oligoarthritis * Inflammation of spine * Inflammation of sacroiliac joints * Stiff neck * Stiff back * Neck pain * Back pain
Causes
The cause of acne is multifactorial, but theories regarding dietary influences appear to be groundless. Predisposing factors include heredity; hormonal contraceptives (many females experience an acne flare-up during their first few menstrual cycles after starting or discontinuing hormonal contraceptives); androgen stimulation; certain drugs, including corticosteroids, corticotropin, androgens, iodides, bromides, trimethadione, phenytoin, isoniazid, lithium, and halothane; cobalt irradiation; and hyperalimentation. Other possible factors are exposure to heavy oils, greases, or tars; trauma or rubbing from tight clothing; cosmetics; emotional stress; and unfavorable climate. More is known about the pathogenesis of acne. (See What happens in acne.) Androgens stimulate sebaceous gland growth and production of sebum, which is secreted into dilated hair follicles that contain bacteria. The bacteria, usually Propionibacterium acnes and Staphylococcus epidermidis (which are normal skin flora), secrete lipase. This enzyme interacts with sebum to produce free fatty acids, which provoke inflammation. Also, the hair follicles produce more keratin, which joins with the sebum to form a plug in the dilated follicle. Acne vulgaris primarily affects adolescents (usually between ages 15 and 18), although lesions can appear as early as age 8. Although acne strikes boys more often and more severely than girls, it usually occurs in girls at an earlier age and tends to last longer, sometimes into adulthood.
Prognosis
The 'prognosis' of SAPHO syndrome usually refers to the likely outcome of SAPHO syndrome. The prognosis of SAPHO syndrome may include the duration of SAPHO syndrome, chances of complications of SAPHO syndrome, probable outcomes, prospects for recovery, recovery period for SAPHO syndrome, survival rates, death rates, and other outcome possibilities in the overall prognosis of SAPHO syndrome. Naturally, such forecast issues are by their nature unpredictable.
Treatment
Treatment for acute osteomyelitis should begin before definitive diagnosis. Treatment includes administration of antibiotics after blood cultures are taken; early surgical drainage to relieve pressure buildup and sequestrum formation; immobilization of the affected bone by plaster cast, traction, or bed rest; and supportive measures, such as analgesics and I.V. fluids. If an abscess forms, treatment includes incision and drainage, followed by a culture of the drained fluid. Intracavitary instillation of antibiotics may be done through closed-system continuous irrigation with low intermittent suction; limited irrigation with blood drainage system with suction; or local application of packed, wet, antibiotic-soaked dressings. In addition to these therapies, chronic osteomyelitis usually requires surgery to remove dead bone (sequestrectomy) and to promote drainage (saucerization). The area may be filled with bone graft or packing material to promote new bone tissue. An infected prosthesis is removed and a new one is implanted the same day or after resolution of the infection. Some centers use hyperbaric oxygen to increase the activity of naturally occurring leukocytes. Free-tissue transfers and local muscle flaps are also used to fill in dead space and increase blood supply.
Resources
Main name of condition: SAPHO syndrome Other names or spellings for SAPHO syndrome: Synovitis [acne - pustulosis - hyperostosis - osteitis], Synovitis acne pustulosis hyperostosis osteitis, synovitis, acne, pustulosis, hyperostosis, osteomyelitis, Acquired hyperostosis syndrome, Synovitis, Acne, Pustlosis, Hyperostosis, and Osteomyelitis Acquired hyperostosis syndrome Source - Diseases Database Acquired hyperostosis syndrome, Synovitis, Acne, Pustlosis, Hyperostosis, and Osteomyelitis Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)