SAT0069 Sapho syndrome

Abstract

Background The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) was first introduced by Chamot et al. in 1987.1 The syndrome is characterised by variable bone involvement, with or without concurrent pustular dermatoses. The affected bones include the chest wall (clavicle, sternum, sternoclavicular joints), sacroiliac joints and long bones. Bone changes include hyperostosis, aseptic osteomyelitis and arthritis. Skin diseases include palmoplantar pustulosis, acne conglobata or acne fulminans and hidradenitis suppurativa. Objectives To determine the frequency of clinical and bone scintigraphic changes in patients with SAPHO syndrome. Methods Authors analyse the clinical and radiological findings and outcome of the disease in 6 cases of SAPHO syndrome (3 males, 3 females) between 1997 and 2000 retrospectively. Results Authors treated 6 cases with SAPHO syndrome between 1997 and 2000 of whom 3 patients suffered from acne fulminans and 3 patients from palmoplantar pustulosis. Acne fulminans was found exclusively in young males (mean age: 20 years) including one patient who suffered from inflammatory bowel disease (ulcerative colitis). Sacroileitis was found in all acne patients, and 2 cases had chest wall arthroosteitis and peripheral arthritis also. The joint involvement was preceded by the acneiform eruption. Treatment with isotretinoin was suspected playing a role in the development of joint involvement in 2 cases. All patients with palmoplantar pustulosis were middle-aged women (mean age: 45 years). All of these 3 patients suffered from anterior chest wall involvement and one had peripheral joint symptoms. The diagnosis of SAPHO syndrome based on clinical symptoms, laboratory findings (elevated erythrocyte sedimentation rate, leukocyte count, and C-reactive protein), and the results of the bone scintigraphy. Bone scintigraphy is an important examination which can reveal the early pathologic signs when the X-ray examinations are still negative. Conclusion Bone scintigraphy is a sensitive examination in the early diagnosis of the SAPHO syndrome. Cases with acne fulminans have more favourable prognosis, whereas cases with palmoplantar pustulosis have a chronic course. References Chamot AM, Benhamou CL, Kahn MF, et al. Synovitis-acne-pustulosis-hyperostosis- osteitis. Results of a national survey 85 cases. Rev Rhum Mal Osteoartic. 1987;54(3):187–96 Gutzner R, Herbst RA, Kapp A, Weiss I. SAPHO syndrome. Case description of 3 patients with acne conglobata and osteoarticular symptoms. Hautarzt 1997;48(3):186–90 Karolyi ZS, Fazekas K. SAPHO syndrome. Borgyogy Vener Szle (Hungary) 1998;74(1):23–6