Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome in Childhood; A Rare Clinical Entity

Ayşe Sayılı*1, MD, Ozgur Tosun2, Nazan Cobanoglu1, Nerin Bahceciler Onder1, Fusun Baba3, Salih Kavukcu1 1Department of Pediatrics, 2Department of Radiology, 3Department of Pathology, Near East University Faculty of Medicine, Turkish Republic of Northern Cyprus Received: Sep 11, 2013; Accepted: Apr 23, 2014; First Online Available: Aug 15, 2014 Dear Editor, Referring to synovitis, acne, pustulosis, hyperostosis and osteitis; SAPHO syndrome is defined as a chronic, relapsing rheumatologic disease of uncertain etiology characterized by distinct osteoarticular and cutaneous manifestations. There have been recent reports of chronic recurrent multifocal osteomyelitis (CRMO) occurring in adults and SAPHO syndrome occurring in children whereas just the vice-versa is normally expected[1,2]. Herein, we would like to emphasize a rare form of SAPHO syndrome in terms of age and the localization of the disease. A 12-year-old female patient was admitted to our hospital with the complaint of right hip and low back pain. She had tenderness on the right iliac crest. She had normal hemogram with an erythrocyte sedimentation rate (ESR) of 51 mm/hour. Serological tests for Salmonella and Brucella were negative. Having a BCG vaccination scar, PPD was negative and no abnormalities were found in chest radiography of the patient. Bone marrow aspiration and bone scintigraphy revealed no signs of malignancy. Anti-streptolysin O, Creactive protein, anti-nuclear anitobody, anti double stranded DNA and HLA B27 were negative. Serum immunoglobulin levels were within the normal range. Magnetic resonance imaging (MRI) (Fig. 1), showed increased density of right acetabular area and surrounding soft tissue besides bone marrow edema. She received 15 mg/kg/day naproxen sodium administered. In a month’s time, regression of complaints occurred while occasional emergence of fever and pain on the left hip was identified. Repeated pelvic MRI (Fig. 2) revealed involvement of head of Fig. 1: Coronal fat suppressed T2 weighted image shows edema in right acetabulum and surrounding soft tissues femur on the left side, L5 and S1 vertebrates characterized with hyper-density and edema. Naproxen sodium treatment was continued for almost next 4 months. She stopped having this treatment on her own accord, and then applied to our hospital with vesiculopustular skin lesions on the palmar, plantar and retroauricular surfaces. Histopathologic findings revealed subcorneal intraepidermal vesicles as well as intracorneal plasma insudation. Lymphocyte exocytosis, infiltrating vesicles and epidermal cells were also visualized. Patient received oral analgesic, nonsteroidal anti-inflammatory drug (Ibuprofen) and topical steroid treatment. Remarkable increase in the intensity of edema signals was notable in the evaluation of serial radiological findings (Fig 3). Naproxen sodium administration was continued and after one month there was a clinical regression in the skeletal system findings and skin lesions. Being in the 18th month, she is still under follow up with no problems occurring to date. Classification of periodic fever syndromes under the auto-inflammatory disorders has been followed by the definition of auto-inflammatory diseases with particular involvement of bone tissue. These are mainly, CRMO, SAPHO syndrome, Majeed syndrome, deficiency of interleukin-1 receptor antagonist (DIRA) and cherubism. SAPHO syndrome is a clinical entity characterized by inflammation in bone, joint and skin. Inflammation manifests in the form of sterile osteomyelitis and hyperostosis in the bone, acne or pustulosis in the