Severe cutaneous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome are rare, and its treatment remains a therapeutic challenge.We report here a patient with SAPHO syndrome with impressive cutaneous manifestations in the form of extensive pyoderma vegetans (PV) and follicular occlusion triad with concurrent chronic hepatitis C, who was treated successfully with etanercept.
CASE REPORTA 47-year-old white male presented with chronic suppurative, malodorous, generalized lesions that impaired his norrnal well-being and caused him profound psychological distress and social incapacity ( Fig. la-d). The lesions had first developed 7 years previously as superficial pustules that evolved into suppurative plaques and nodules, eventually healing and leaving scars. New crops of lesions developed periodically, with occasional febricula and chills. Long-term, high doses of oral antimicrobials provided some relief. He had also had acne conglobata on back and nape, hidradenitis suppurativa (Fig. le), and perifolliculitis abscedens et suffodiens since his teens, which had been treated with oral antibiotics and isotretinoin with poor results. In addition, 2 years previously the patient had begun to develop progressive polyarticular seronegative arthritis with axial and peripheral manifestations, diagnosed on clinico-radiological grounds, consisting of synovitis of the right wrist and elbow, pain in the sole consistent with enthesopathy and HLA-B27-negative sacroileitis. Articular symptoms correlated with flare-ups of cutaneous manifestations. He was treated with salazopyrin, non-steroidal anti-inflammatory drugs (NSAIDs), and six infusions of pamidronate, with poor results. Finally, he had also had an untreated chronic hepatitis C virus (HCV) infection for an unspecified length of time.Extensive laboratory evaluations revealed only leukocytosis, raised erythrocyte sedimentation rate (ESR) (72 mm/h, normal 0-20) and raised transaminases (ALT: 178 U/L, normal 5^1; AST 147 U/L, normal 5-38). Notably, iodide and bromide levels were within the normal range and anti-neutrophil cytoplasmic antibodies (ANCAs) were negative. Biopsy of a recent pustular lesion showed a superficial suppurative folliculitis. Biopsy of an evolutionated lesion showed pseudoepitheliomatous and infundibular hyperplasia, multiple abscesses and fistulization. Cultures of repeated purulent discharge disclosed the growth of Proteus penneri and Staphylococcus aureus in an 18-month follow-up period despite the absence of any form of treatment. Skin biopsy culture, searching for slow-growing microorganisms, was negative.In view of these features we established a diagnosis of PV(1) in the context of SAPHO syndrome (2). At the time of diagnosis the patient had a high score of 23 on the Dermatology Life Quality Index (DLQI; normal 0-3, maximum 30) (3).A 12-month cycle of oral cyclosporine produced moderate control of the lesions (DLQI: 15), but was discontinued due to an important flare-up of the disease (DLQI: 23). Finally, he started s...