Sapho syndrome M86.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 15.07.2022

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Sonozaki, 1981; Chamot AM,1987 (coined the acronym SAPHO)

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SAPHO is the acronym for:

  • Synovitis
  • Acne
  • Pustulosis
  • Hyperostosis
  • Osteitis.

Clinically multifaceted autoinflammatory skin-bone-associative syndrome (SkoBo) occurring in all age groups with a mostly recurrent , subacute to chronic course. The most common partial manifestation of the syndrome is chronic, recurrent (non-bacterial) multifocal osteomyelitis, which may also involve adjacent joints. Comorbid, landmark, dermatologic monitor symptoms in this case are the following neutrophilic dermatoses:

SAPHO syndrome is also referred to as "skibo-disease" ("skin and bone disease") to emphasize the characteristic association of pathologic osteoarticular and dermatologic symptoms.

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The etiology of SAPHO syndrome is not fully understood. The osteo-articular disease processes include a probable reactive-immunologic (rheumatoid factor-negative), oligo- or polytopic, inflammatory but never septic (non-bacterial) process of the bone and adjacent joint structures with inflammatory enthesiopathies. A possible trigger function proceeds from skin infections, although intervals of one year may lie between the dermatological and osteo-articular manifestations .

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No sex emphasis. Skin lesions can manifest themselves 30% at juvenile age and 70% at adult age.

Clinical features
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The criteria for this syndrome are defined as sterile osteitis (blanched, non-bacterial osteomyelitis) including spondylitis and/or arthritis, which may occur with or without skin lesions. SAPHO is now more commonly understood as an umbrella syndrome and overarching term for a number of syndromes and disease entities, esp:

Within the umbrella syndrome SAPHO, various associations and overlaps are possible, with the cutaneous-osseous association not being an obligate but a characteristic phenomenon. Also described is an enteropathic variant of SAPHO syndrome that, in addition to the above symptoms, is associated with Crohn's disease or ulcerative colitis (approximately 5% of SAPHO patients (Naves JE et al. 2013). Of note and etiopathogenetic interest is the occurrence of SAPHO syndrome during isotretinoin therapy of acne fulminans (Luzzati M et al. 2020)

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Autoantibodies were found in a larger SAPHO collective in about 22% of patients: 15.5%: positive ANA (titer >/= 1/160); antithyroid antibodies (anti-TPO and/or anti-Tg antibodies): Antibodies against gastric parietal cells: 3.3 %, antibodies against smooth muscle:4.4 %. Antimitochondrial and LKM antibodies: negative. Anti-CCP and anti-dsDNA antibodies: negative (Grosjean C et al. 2010). To what extent an etiopathogenetic significance must be attributed to them is open.

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Skin histological picture of Pustulosis palmaris et plantaris with unilocular pustule in the area of the surface epithelium.

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There are numerous, but not standardized, modalities of therapy.

  • Initial: Non-steroidal anti-inflammatory drugs. If necessary, in the acute painful stage, systemic glucocorticoids in mttler dosage (e.g. prednisolone 50 mg/day p.o.), fumaric acid esters (with or without methotrexate; MTX dosage: 15-25 mg/week p.o.).
  • Also effective are TNF-alpha blockers (e.g., infliximab or etanercept).
  • Systemic antibiotics are usually ineffective.
  • In elderly patients with significant osteopathic pain, bisphosphonates such as pamidronate (single application of 60 mg i.v. every 4 months) or zoledronic acid (single administration of 4 mg i.v.) have been used successfully.

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SAPHO syndrome belongs to the extended group of so-called autoinflammatory diseases. Pathogenetically, a relationship or identity of the SAPHO syndrome with the pustular arthroosteitis described by Sonozaki and the chronic recurrent multifocal osteomyelitis (CRMO) of childhood is discussed. The sterno-costo-clavicular hyperostosis (ACW -so-called anterior chest wall syndrome) is a polyetiological, reactive symptom complex associated with pustulosis palmaris et plantaris on the skin.

Case report(s)
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1) Reported is the 8-year treatment course of a 40-year-old man with hidradenitis suppurativa and synovitis, acne, pustulosis palmaris et plantaris, hyperostosis, and osteitis syndrome. Initial oral and topical antibiotics had little effect. Intralesional corticosteroid injections were effective for localized inflammatory lesions but were not sufficient to control hidradenitis suppurativa. Introduction of adalimumab and local remediation of hidradenitis suppurativa resulted in stabilization. Adalimumab resulted in dramatic improvement in back pain. Continuation of treatment with adalimumab and subsequent administration of methotrexate resulted in extensive improvement of the clinical picture.

2) The case of a boy who suffered from acne fulminans and depression and developed sacroiliitis after 10 weeks of treatment with isotretinoin is presented. After SAPHO diagnosis, NSAID therapy was initiated, but the appearance of bilateral gluteal hidradenitis suppurativa required a switch to a TNF-alpha antagonist(adalimumab), with which good control of the disease was achieved. Despite specific therapy with sertraline, the patient continued to complain of severe depression (Luzzati M et al 2020).

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  1. Chamot AM et al (1987) Acne-pustulosis-hyperostosis-osteitis-syndrome. Results of a national survey . 85 cases. Rev Rhum Mal Osteoartritic 54: 187-196.
  2. Gmyrek R et al (1999) SAPHO syndrome: report of three cases and review of the literature. Cutis 64: 253-258
  3. Grosjean C et al (2010) Prevalence of autoantibodies in SAPHO syndrome: a single-center study of 90 patients. J Rheumatol37:639-43.
  4. Just A et al (2010) Bisphosphonates as a successful treatment principle for primary chronic osteomyelitis in SAPHO syndrome. JDDG 8: 657-550
  5. Kirchhoff T et al (2003) Diagnostic management of patients with SAPHO syndrome: use of MR imaging to guide bone biopsy at CT for microbiological and histological work-up. Eur Radiol 13: 2304-2308
  6. Köhler H, Uehlinger E, Kutzner J et al (1975) Sterno-costo-clavicular hyperostosis. Dtsch med Wschr 100: 1519-1523
  7. Liu S et al (2020) Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update. Ther Adv Musculoskelet Dis 12:1759720X20912865.
  8. Luzzati M et al. (2020) SAPHO syndrome: the supposed trigger by isotretinoin, the efficacy of adalimumab and the specter of depressive disorder: a case report. Ital J Pediatr 46:169.
  9. Naves JE et al (2013) A systematic review of SAPHO syndrome and inflammatory bowel disease association. Dig Dis Sci 58:2138-2147.
  10. Ramautar AI et al. (2021) Chronic nonbacterial osteomyelitis of the sternocostoclavicular region in adults: A single-center Dutch cohort study. JBMR Plus 5:e10490.
  11. Schilling F, Kessler S (2000) The SAPHO syndrome: clinical - rheumatologic and radiologic differentiation and classification of a case series of 86 cases. Z Rheumatol 59: 1-28
  12. Sonozaki H et al (1981) Clinical features of 53 cases with pustulotic arthroosteitis. Ann Rheum Dis 40: 547-553
  13. Vekic DA et al (2018) SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate: a case report and review of the literature. Int J Dermatol 57:10-18.
  14. Wohl Y et al (2003) Stress in a case of SAPHO syndrome. Cutis 71: 63-67
  15. Yamasaki O et al (2003) A case of SAPHO syndrome with pyoderma gangrenosum and inflammatory bowel disease masquerading as Behcet's disease. Adv Exp Med Biol 528: 339-341


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Last updated on: 15.07.2022