Synovitis, acne, impetigo, hyperostosis, osteomyelitis complex
Introduction
Synovitis, acne, impetigo, bone hypertrophy, osteomyelitis Synovitis, acne, impetigo, bone hypertrophy, osteomyelitis syndrome (synovitis-acne-pustulosis-hyperostosis-osteomyelitissyndrome), referred to as SAPHO syndrome. SAPHO is an abbreviation for the following five English words: synovitis, acne, pustulosis, hyperostosis, and osteomyelitis. SAPHO syndrome is a chronic disease that mainly affects the skin, bones and joints. The disease mainly includes bone lesions and palmoplantar pustulosis. Sasaki first described a clavicular aseptic hypertrophic osteitis with palmoplantar pustulosis in 1967, and more than 250 cases have been reported since then. In 1987, Charnot et al. summarized the initials of each lesion, proposed the name of SAPHO syndrome, and classified the bone and joint lesions of this syndrome into rheumatoid arthritis associated with hemorrhoids (clumps, outbreaks, sweat glands). , purulent), palm, sputum impetigo, chest clavicular hypertrophy and chronic recurrent osteomyelitis. All patients involved sternal joints and caused aseptic osteotrophic osteitis. basic knowledge Probability ratio: Susceptible people: no special people Mode of infection: non-infectious Complications: psoriasis ankle arthritis
Cause
Synovitis, acne, impetigo, bone hypertrophy, comprehensive cause of osteomyelitis
(1) Causes of the disease
The cause of this disease is unknown, there are several hypotheses:
1. Circulating immune complexes found circulating immune complexes in fulminant acne, which may be the result of an immune response to P. acnes. These immune complexes sink into the bone causing an inflammatory process leading to clinically osteolytic lesions. And some people found P. acnes in about half of the affected joint biopsy specimens.
2. Environmental factors The incidence of pustulosis in some areas is high and is considered to be related to environmental factors.
3. About 1/3 of patients are HLA-B27 positive. Among the families of patients with SAPHO syndrome, 14% had palmoplantia pyoderma, 19% had psoriasis, and 5% had acne.
(two) pathogenesis
At present, the pathogenesis of this disease is still not very clear, and may be related to HLA-B27 positive, genetic family. Others believe that it is associated with prostaglandins. Ueda reported that two infants were treated with prostaglandin E1 for a long time to close the patent ductus arteriosus, and both patients developed cortical hypertrophy, especially the long bones and ribs of the limbs. The bone changes were improved after stopping treatment. Therefore, prostaglandins produced in inflammation may play a role in the pathogenesis of bone hypertrophy.
Prevention
Synovitis, acne, impetigo, bone hypertrophy, osteomyelitis comprehensive prevention
1. Eliminate and reduce or avoid the disease factors, improve the living environment, improve the development of good habits, prevent infection, pay attention to food hygiene, and rational diet.
2. Pay attention to exercise, increase the body's ability to resist disease, do not fatigue, excessive consumption, quit smoking and alcohol. The law of life, work and rest, comfortable, avoid strong mental stimulation.
3. Early detection and early diagnosis and early treatment, establish confidence in the fight against disease, adhere to treatment.
4. Non-steroidal anti-inflammatory drugs can alleviate symptoms. In addition, colchicine and sulfasalazine can also be tried. Care should be taken to prevent skin infections. Severe acne and pustules can be tested with vitamin A.
Complication
Synovitis, acne, impetigo, bone hypertrophy, osteomyelitis Complications Psoriasis Ankle Arthritis
Can be complicated by sterno-arthritis, thoracic rib arthritis, ankle arthritis, psoriasis.
Symptom
Synovitis, acne, impetigo, bone hypertrophy, osteomyelitis syndrome Common symptoms Joint pain Joint pain, joint swelling and pain
The age of onset is mostly young and middle-aged. The age of onset is at least 10 years old and the maximum is 59 years old. The ratio of male to female is different. Patients often have bone and joint swelling and pain, most commonly involved in the sterno-lock joint, thoracic rib joint, shoulder joint, tibia, pubic bone and so on. The sternum was 62%, followed by the ankle (33%), the spine (24%), the surrounding bone (19%), and the surrounding joint (10%). 81% of patients have more than two lesions, and may be accompanied by inflammation around the joints. 52% to 66% of patients have palmoplantia pyoderma, 14% to 15% of patients have hemorrhoids, and 9% to 24% of patients have psoriasis. Skin lesions can occur before or after osteoarthrosis.
The main symptoms are pain and swelling of the anterior chest wall, often bilateral, exacerbated when the weather is wet and cold. After a long course of disease, the thoracic rib clavicular junction is fused, and the bone hypertrophy can compress the adjacent neurovascular structure, sometimes requiring surgical treatment. Laboratory tests are usually non-specific. Bone lesions can also occur in the spine, long bones, tibia, mandible, and pubis. Damage often begins with the attachment of tendons and ligaments, while the latter two also have bone hypertrophy, often with arthritis, especially sacroiliitis. Aseptic damaging lesions (chronic recurrent multifocal osteomyelitis) can be seen in children and young adults, with sclerosis and hypertrophy after healing.
Skin damage can be manifested as palmoplantar pustulosis, suppurative sweat gland inflammation or severe acne (party acne or fulminant acne).
Examine
Comprehensive examination of synovitis, acne, impetigo, bone hypertrophy, osteomyelitis
C-reactive protein is elevated and rheumatoid factor is negative. White blood cell count and blood cell count increased, ANA was positive, and HLA-B27 was about 30% positive. X-ray examination: There was no obvious change in the early stage. As the disease progressed, the sterno-lock joint and the thoracic rib joint were irregularly eroded, and the cortical bone was thick. Can also affect the wrist, neck, chest, waist, sputum, showing the adjacent 2 to 4 vertebral diffuse hyperplasia. Ankle joint lesions are often asymmetrical.
Diagnosis
Comprehensive diagnosis of synovitis, acne, impetigo, bone hypertrophy and osteomyelitis
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
However, attention should be paid to the identification of psoriatic arthritis, ankylosing spondylitis, reiter syndrome and sclerosing osteomyelitis. Some people think that although spondyloarthropathy has many similarities with SAPHO syndrome, the two should be distinguished. The symptoms of SAPHO syndrome are osteitis and cirrhosis, not true arthritis. Ankle arthritis is about 50% unilateral in SAPHO syndrome, and is usually bilateral in spinal joint disease. Osteomyelitis is common in SAPHO syndrome and is not common in spondyloarthropathy.
