Acronym for Synovitis, Acne, Pustulosis, Hyperostosis, Osteomyelitis

Prevalence estimated at 1/10,000 in France. Autoinflammatory disease that combines a cutaneous neutrophilic involvement and a chronic osteomyelitis, resulting in a wide spectrum of abnormalities characterized by osteoarticular and cutaneous manifestations of varying severity.

Onset between childhood and late adulthood, with a median age between 30 and 40 years of age.

In children,

• the osteoarticular involvement is often distributed in a similar way as recurrent chronic multifocal osteomyelitis (in the long bones, clavicle, spine). When synovitis occurs, it most often occurs in the sacroiliacjoints, hips or knees, or in the sternoclavicular joints.
• the cutaneous involvement includes severe acne, palmoplantar pustulosis and pustular psoriasis. Skin manifestations often begin one to two years before bone changes, but they can also appear simultaneously, or more than 20 years later.

Patients may also suffer from abdominal pain, diarrhea, fissures or abscesses, suggesting possible association with inflammatory bowel disease (IBD) (see this term) such as Crohn's disease or ulcerohemorrhagic colitis.

Recurrent chronic multifocal osteomyelitis is often considered as a pediatric form of SAPHO syndrome. SAPHO syndrome is clinically similar to  the syndrome of the Interleukin 1 Receptor Antagonist deficiency (DIRA) (see this term) of genetic origin.

Treatments: NSAIDs or corticosteroids, sometimes methotrexate.

Treatment of skin involvement: azithromycin, PUVA therapy or (phase 3): tofacitinib (Janus kinase inhibitor) 5 mg 2x/day.

Anesthetic implications:

fragile skin, antibiotic prophylaxis, drug interactions due to treatment (corticosteroids)

References : 

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Updated: January 2021