The SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare, chronic, autoinflammatory, debilitating disease. The average age of onset is between 30 and 40 years. The etiology is multifactorial,… Click to show full abstract
The SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare, chronic, autoinflammatory, debilitating disease. The average age of onset is between 30 and 40 years. The etiology is multifactorial, including environmental, genetic, immunological, or infectious causes like Propionibacterium acnes. SAPHO syndrome can be associated with other neutrophilic disorders. Hidradenitis suppurativa (HS), or acne inversa, is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle. It usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body. A few cases of concomitant SAPHO and HS have been described. Some of these cases presented occasional ocular involvement, including bilateral keratitis, acute anterior uveitis, persistent proteinuria, and tumors. The quality of life of patients affected by both SAPHO and HS could be very compromised and, often, treatment of both diseases could be challenging. We report the case of a 22-year old man who came to our attention for severe acne of the trunk and neck, and multiple nodules, abscesses, and tunnels localized in the armpit, groin, back, and chest (Figure 1 and 2, A-C), arisen 2 years earlier, and compatible with the diagnosis of HS. For this condition the patient was previously treated with various antibiotics (ie, penicillin, tetracycline, clindamycin and rifampicin), and several drainages of the cutaneous lesions, with scarce improvement. Six years earlier, he had received the diagnosis of acne fulminans and he was treated for 6 months with methylprednisolone 25 mg/day and isotretinoin 30 mg/day, with resolution of the disease, while a mild acne persisted on the face. One year later, the patient was diagnosed with SAPHO syndrome, during a rheumatology consultation, due to diffuse arthralgia. MRI (Figure 3A,B) showed osteitis and hyperostosis of the sternoclavicular joints, and bone scintigraphy (Figure 3C) revealed intense hyperactivity of the sternoclavicular and sacroiliac joints and of the C4 soma. For the SAPHO syndrome, he was previously treated in different periods with cyclosporine 200 mg/day for 2 months, Adalimumab 40 mg weekly for 2 months, and then with Infliximab 5 mg/kg for 3 months, with mild improvement of the disease. When he came to our attention, besides SAPHO syndrome and HS, the patient had no comorbidities. In that time, he was treated with Adalimumab 80 mg weekly, Methotrexate 10 mg weekly, metformin 1 g/day, and prednisone 5 mg/day. Considering the good control of the rheumatic disease, but given the flare-ups of the HS, mainly localized on the armpit, we decided to inject the inflamed lesions of this region with triamcinolone 40 mg plus lincomycin 600 mg diluted in saline solution for a total volume of 20 mL, three times at 2-week intervals. A stark improvement was
               
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