SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics. An entity initially known as chronic recurrent multifocal osteomyelitis was first described in 1972. Subsequently, in 1978, several cases of were associated with blisters on the palms and soles ( palmoplantar pustulosis). Since then, a number of associations between skin conditions and osteoarticular disorders have been reported under a variety of names, including sternocostoclavicular

hyperostosis Hyperostosis is an excessive growth of bone. It may lead to exostosis. It occurs in many musculoskeletal disorders and from use of drugs like Isotretinoin. Disorders featuring hyperostosis include: * Camurati-Engelmann disease, type 2 * Hyper ...

, pustulotic arthro-osteitis, and acne-associated

spondyloarthropathy Spondyloarthritis (SpA), also known as spondyloarthropathy, is a collection of syndromes connected by genetic predisposition and clinical symptoms. The best-known subtypes are enteropathic arthritis (EA), psoriatic arthritis (PsA), ankylosing ...

. The term SAPHO (an acronym for

synovitis Synovitis is the medical term for inflammation of the synovial membrane. This membrane lines joints that possess cavities, known as synovial joints. The condition is usually painful, particularly when the joint is moved. The joint usually swells ...

arthritis Arthritis is a general medical term used to describe a disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, Joint effusion, swelling, and decreased range of motion of ...

, pustulosis,

osteitis Osteitis is inflammation of bone. More specifically, it can refer to one of the following conditions: * Osteomyelitis, or ''infectious osteitis'', mainly ''bacterial osteitis'' * Alveolar osteitis or "dry socket" * Condensing osteitis (or Osteit ...

) was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.

Diagnosis

Radiologic findings

* Anterior chest wall (most common site, 65–90% of patients): Hyperostosis, sclerosis and bone hypertrophy especially involving the sternoclavicular joint, often with a soft tissue component. * Spine (33% of patients): Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement. * Long bones (30% of patients): usually metadiaphyseal and located in the distal femur and proximal tibia. It looks like chronic osteomyelitis but will not have a sequestrum or abscess. * Flat bones (10% of patients): mandible and ilium. Peripheral arthritis has been reported in 92% of cases of SAPHO as well. In children, the SAPHO syndrome is most likely to affect the

metaphysis The metaphysis (: metaphyses) is the neck portion of a long bone between the epiphysis and the diaphysis. It contains the growth plate, the part of the bone that grows during childhood, and as it grows it ossifies near the diaphysis and the ep ...

of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.

Treatment

Bisphosphonate therapy has been suggested as a first-line therapeutic option in many case reports and series. Treatment with tumor necrosis factor alpha antagonists (

TNF inhibitor Tumor necrosis factor (TNF), formerly known as TNF-α, is a chemical messenger produced by the immune system that induces inflammation. TNF is produced primarily by activated macrophages, and induces inflammation by binding to its receptors o ...

s) has been tried in a few patients with limited success. Other drugs that are used in

psoriatic arthritis Psoriatic arthritis (PsA) is a long-term inflammatory arthritis that may occur in some people affected by the autoimmune disease psoriasis. The classic features of psoriatic arthritis include dactylitis (sausage-like swelling of the fingers ...

, to which SAPHO syndrome is closely related, have also been used in this condition. They include NSAIDs,

corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...

, sulfasalazine,

methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...

ciclosporin Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is taken Oral administration, orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease, nephr ...

and

leflunomide Leflunomide, sold under the brand name Arava among others, is an immunosuppressive disease-modifying antirheumatic drug (DMARD), used in active moderate-to-severe rheumatoid arthritis and psoriatic arthritis. It is a pyrimidine synthesis inhib ...

. Some patients have responded to

antibiotics An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy ...

. The rationale for their use is that Cutibacterium acnes, a bacterium known for its role in acne, has been isolated from bone biopsies of SAPHO patients.

References

External links

{{Disorders of skin appendages Ailments of unknown cause Acneiform eruptions Rheumatology Syndromes

Diagnosis

Radiologic findings

Treatment

See also

References

Further reading

External links