Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

Abstract

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is defined as a syndrome that is related to various osteoarticular manifestations and chronic dermatological conditions especially severe acne. SAPHO syndrome is a rare and unusual clinical entity in childhood and treatment choices are variable. We report an 11-year-old girl who suffered from SAPHO syndrome and successfully treated with subcutaneous methotrexate. We report our case in order to take attention to this rare clinical condition in evaluating patients and also to point out that treatment options beyond biologic agents should be the first line treatment in childhood.Key words: children, methotrexate, SAPHO syndrome, treatment.The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) represents a syndrome characterized by the variable association of osteoarticular manifestations and various chronic dermatological conditions, particularly palmoplantar pustulosis and severe acne 1 . Although there have been case series of adults who have been followed up by the diagnosis of SAPHO syndrome 2,3 , there are few cases in childhood as case presentations 4,5 . The diagnosis of the SAPHO syndrome is based on exclusion of infectious arthritis, osteomyelitis, and tumoral conditions of the bone; and the presence of at least one of four diagnostic criteria proposed by Benhamou et al 6 .Here, we present an adolescent girl who was diagnosed as SAPHO syndrome and has been followed up for two years in remission with subcutaneous methotrexate therapy. Case ReportAn 11-year-old girl admitted to our hospital with a history of pain on her left thigh, both ankles, left hip, spine and on sternum especially when taking breaths for 20-days and swelling on both ankles for two days. She was not even able to move or walk due to severe pain. She had lost 4 kg during the last 3 weeks. She did not have fever or any trauma. Family history and past medical history was unremarkable. Physical examination revealed pustuloform acne on the face, and pustular lesions on the whole body ( Fig. 1), Sternal tenderness, ankle swelling and reduced motility of the vertebral column were remarkable. In her laboratory evaluation, erythrocyte sedimentation rate was 105 mm/ hour and high sensitive CRP was 6.23 mg/dl (0-0.2 mg/gl). All other rheumatologic parameters including rheumatoid factor and autoantibodies were within normal ranges or negative. She was negative for HLA-B27. Full septic screens were negative. Cultures of the pustular lesions were negative. Conventional X-rays showed periostal reactions on the head of femur and osteitis on the bilateral sacroiliac bones. MRI showed pathologic signals and contrast uptake on L1 vertebra, sacrum, left sacroiliac bones and the adjacent soft tissues suggesting malignancy (Fig. 2, 3). Nuclear bone scans demonstrated

SAPHO syndrome with pathological fractures of vertebral bodies: a case report

“…NSAIDs are the first-line treatment. Several studies have supported the effectiveness of bisphosphonates, steroids, methotrexate, tumor necrosis factor (TNF) inhibitors and so on as effective treatments [16–18]. While before we treated this patient, several reports had shown that some patients may develop or have paradoxical worsening of skin lesions after starting TNF inhibitors with unclear reasons [19, 20], which was confirmed by a recent array research [21].…”

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

“…For patients with peripheral joint involvement and relatively low levels of axial spine joint involvement, the administration of methotrexate (MTX) could be effective. 72–74 Improvement of peripheral osteoarticular symptoms and pleural effusions could occur within several weeks to months. However, the efficacy of MTX for the treatment of osteitis, osteomyelitis or enthesitis is uncertain.…”

Pediatric Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) Syndrome: Diagnostic Challenges and Treatment Approach

“…Akçaboy et al reported a case of an 11-yearold girl, who suffered from SAPHO and, after an unsuccessful combination of NSAIDssulfasalazine and prednisolone, was relieved by administration of subcutaneous methotrexate. At a two-year follow-up, she was free of symptoms [13].…”