SAPHO SYNDROME

Palmoplantar pustulosis as part of the SAPHO syndrome


The SAPHO syndrome is the association of Synovitis, Acne, Pustulosis, Hyperosteosis and Osteitis. It represents a multi systemic inflammatory reaction, at times to the chronic infection by P. acnes.

Clinical

Palmoplantar pustulosis is the most typical cutaneous finding (estimated at 65% of cases by one source) followed by severe acne, e.g. acne fulminans or acne conglobata. Hidradenitis suppurativa, psoriasis, pyoderma gangrenosum and Sweet's syndrome have also been reported. With regard to bony pain, anterior chest wall tenderness is most common. Children and adults less than 60 years of age are most commonly affected. The skin lesions often precede the bony abnormalities.

Treatment

NSAIDs are often used for pain control along with intraarticular steroid injections. Methotrexate, sulfasalazine, biphosphonates, etanercept and infliximab have been used. Anakinra showed benefit in 5/6 patients in one study [Ann Rheum Dis 2012;71;1098].

Antibiotics

Antibiotics may be effective because of the association of SAPHO syndrome with P. acnes infection. Azithromycin therapy resulted in significant improvement in one study, but the improvement was lost after the therapy was discontinued. The dose used was azithromycin 500 mg/day x 5 days followed by 500 mg twice a week for 3 months. In that same study, 14/21 patients who underwent bone biopsy and culture grew out P. acnes. (Arthritis Research.com) Other antibiotics used include doxycycline and clindamycin. This author has had one patient who did okay on azithromycin 500 twice a week, but significantly better on 500 three times a week.

In article about combating P. acnes infections of implants [Biomed Res Int. 2013;2013:804391], the following were recommended for that: "Intravenous penicillin G and ceftriaxone are the first choice for serious infections, with vancomycin and daptomycin as alternatives, and amoxicillin, rifampicin, clindamycin, tetracycline, and levofloxacin for oral treatment".

Additional Pictures

50ish year old man who presented with classic pyoderma gangrenosum of the hands and fingers, and later palmar plantar pustulosis, pus in surgical sites (pictured) and pus under the nails. All were standard culture negative (not tested for P. acnes).

Palmoplantar pustulosis as part of the SAPHO syndrome Palmoplantar pustulosis as part of the SAPHO syndrome Palmoplantar pustulosis as part of the SAPHO syndrome Pus emenating from a surgical site as a manifestation of the SAPHO syndrome

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