SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare condition describing the combination of inflammatory skin, bone and joint manifestations. SAPHO syndrome has a heterogeneous presentation that results in diagnostic difficulties. The limited understanding of underlying pathogenesis, variable response to empiric treatment mainly aimed at symptom management, and the chronic relapsing nature of the disease has resulted in insufficient therapeutic results for many patients. Newer treatment options such as tumour necrosis factor alpha (TNFa) antagonists have provided effective alternatives to therapy in this population.
We report a case of severe SAPHO syndrome in association with hidradenitis suppurativa and pyoderma gangrenosum in a 27-year-old male treated successfully with Adalimumab and Methotrexate. The patient had an initial migratory arthritis affecting the knees, ankles, metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulder, and lower back. This progressed to a persistent arthritis and swelling at the sternum, shoulders, wrists, hands, feet and lower back. Radiographic changes were consistent with the diagnosis of SAPHO syndrome. Serum proinflammatory cytokine levels were significantly elevated: TNFa 941.3, interleukin 1beta (IL-1b) 929.8, IL-8 181.5, and IL-17 161.2 levels. Three months post therapy these had fallen to TNFa 274.8, IL1b 52.1, IL8 238.8, and IL17 22.7.
SAPHO syndrome presents a therapeutic challenge for Dermatologists. TNFa antagonists have shown success in management of SAPHO syndrome in case reports, most frequently with use of Infliximab. Methotrexate is shown to provide an excellent result in patients with peripheral arthritis. Our case demonstrates clinical and biological improvement with subcutaneous TNFa therapy and methotrexate.

Dr. Dunja Vekic

Ingham Institute for Applied Medical Research; Department of Dermatology Liverpool Hospital