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Pustulotic arthro-osteitisM13.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.07.2022

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Synonym(s)

Acquired hyperostosis syndrome; AHS; Anterior Chest Wall Syndrome; Arthroosteitis; Arthroosteitis juxtasternale; Hyperostosis; Hyperostosis sterno-costoclavicular; Hyperostosis Syndrome; Hyperostosis syndrome acquired; intersterno-costoclavicular ossification; Ossification intersterno-costoclavicular; Sonozaki Syndrome; spondylarthritis hyperostotica pustulo-psoriatica; sterno-costoclavicular ossification

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HistoryThis section has been translated automatically.

Köhler et al. 1975; Sonozaki 1981

DefinitionThis section has been translated automatically.

Nonspecific, reactive hyperostosis of the adult with productive spondylopathy in the region of the sternoclavicular joints and the upper rib cartilages, which is frequently accompanied by pustulosis palmo-plantaris or psoriasis pustulosa palmo-plantaris. Most likely an abortive manifestation form of psoriatic arthritis.

Rare association of pustulosis palmaris et plantaris or psoriasis pustulosa palmaris et plantaris with ostitic and arthritic changes of the sternoclavicular, upper sternocostal and/or manubriosternal joints (so-called anterior chest wall syndrome), first described in Japan. Leads to extensive peri- and hyperostosis of the anterior thoracic skeleton and eventually possibly to bony ankylosis.

EtiopathogenesisThis section has been translated automatically.

Unknown. Pathogenetically, a relationship of pustular arthroosteitis with chronic recurrent multifocal osteomyelitis of childhood(CRMO) is discussed. Sterno-costo-clavicular hyperostosis is an etiologically as well as pathogenetically unclear symptom complex. In Japan, a cluster with pustulosis palmo-plantaris has been described (see also below SAPHO syndrome).

Clinical featuresThis section has been translated automatically.

Palmoplantar circumscribed partly vesicular, partly pustular skin lesions on reddened hyperkeratotic plaques(pustulosis palmaris et plantaris); pain and swelling in the sternoclavicular region and on the upper costal cartilages, occasionally local skin redness. Intensification of symptoms by cold, wetness or trivial infections.

LaboratoryThis section has been translated automatically.

Non-specific signs of inflammation that fluctuate with the activity of the process.

HistologyThis section has been translated automatically.

Depending on activity, image of a non-specific inflammatory process, always without detection of bacteria, with signs of increased bone remodeling with excess bone remodeling.

Differential diagnosisThis section has been translated automatically.

Bacterial osteomyelitis; osteomyelitis in acne fulminans; Tietze syndrome.

External therapyThis section has been translated automatically.

Therapy of pustulosis palmaris et plantaris or psoriasis pustulosa palmaris et plantaris, see there. Initially mostly glucocorticoids under occlusion, later, after the onset of the systemic effect of internal therapeutics, local therapy with e.g. urea-containing external agents (e.g. basodexan); if necessary, mild tar-containing formulations, e.g. 5% LCD cream R153.

Internal therapyThis section has been translated automatically.

Acute arthritic symptoms require systemic therapy depending on the degree of symptomatology:

  • Methotrexate: Drug of 1st choice. Individual weekly ED, depending on the severity of the findings 5-25 mg methotrexate/week i.m., dose reduction according to clinical findings. 24 h after application of methotrexate substitution with folic acid in the dosage of the previously administered methotrexate. Caveat. In case of hepatotoxicity and bone marrow depression, if necessary, 36 h after methotrexate administration 9 mg leucovorin i.m.
  • Acitretin (Neotigasone): Initial 0.5-1.0 mg/kg bw/day, maintenance dose at 0.5 mg/kg bw/day or lower. Caveat. Teratogenic effect, not in women of childbearing age.
  • Ciclosporin A (e.g., Sandimmune): Initially 2.5 mg/kg bw/day, increase max to 5.0 mg/kg bw/day, after improvement of symptoms slowly reduce dose in steps of 0.5 mg/kg bw/day every 14 days until individual maintenance dose. See below for dosage, NW, AI, and local therapy. Psoriasis vulgaris.
  • TNF-alpha blockers

Progression/forecastThis section has been translated automatically.

Years of course with exacerbations and remissions of inflammatory symptoms. Possible thrombosis of the subclavian vein due to intrathoracic extension of the hyperostosis with congestion of the vein under influence; spontaneous fractures with pseudarthrosis. Roentgen: Sterno-costoclavicular hyperostosis which affects the medial parts of the clavicles, the uppermost ribs of the manubrium sterni. Local soft tissue ossification including the ligamentum costoclaviculare. Bony ankylosis of the sternoclavicular joints. Scintigraphic: Increased accumulation of radionucleotides as an indication of increased bone remodeling.

LiteratureThis section has been translated automatically.

  1. Dihlmann W, Hering L, Bargon GW (1988) The acquired hyperostosis syndrome (AHS). Fortschr Röntgenstr 149: 386-391
  2. Gmyrek R et al (1999) SAPHO syndrome: report of three cases and review of the literature. Cutis 64: 253-258
  3. Ikegawa S et al (1999) Three cases of pustulotic arthro-osteitis associated with episcleritis. J Am Acad Dermatol 41: 845-846.
  4. Jang KA et al (1998) Four cases of pustulotic arthro-osteitis. J Dermatol 25: 201-204
  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. Ramautar AI et al (2021) Chronic nonbacterial osteomyelitis of the sternocostoclavicular region in adults: A single-center Dutch cohort study. JBMR Plus 5:e10490
  8. 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
  9. Schilling F (1991) Pustular arthroosteitis. Dermatol 42: 194-197
  10. Sonozaki H et al (1981) Clinical features of 53 cases with pustulotic arthroosteitis. Ann Rheum Dis 40: 547-553
  11. Vaccaro M et al (2001) Successful treatment of pustulotic arthro-osteitis (Sonozaki syndrome) with systemic cyclosporine. Clin Exp Dermatol 26: 45-47
  12. Wohl Y et al (2003) Stress in a case of SAPHO syndrome. Cutis 71: 63-67
  13. 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

Authors

Last updated on: 09.07.2022

Author: Prof. Dr. med. Peter Altmeyer