Spondarthritis hyperostotica pustulo-psoriatica - Review and a clinical-radiological study with special emphasis on SAPHO syndrome.

Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp - Schilling), corresponding nosologically to pustulotic arthroosteitis, is a dermato-ske letal "double system" disease of adults. It consists of the triad (a) palmo -plantar pustulosis (Ppp) or, alternatively, Konigsbeck-Barber-type psorias is, (b) sternocostoclavicular hyperostosis (SCCH), and (c) truncal-skeletal changes with syndesmophite-like, hyperostotic and/or parasyndesmophite-lik e ossifications of layers of the anterior vertebral ligament taken together in the sense of a desmophytal hyperostosis. There is also a potential for sclerosing inflammatory arthritis of the sacro-iliac joints and "dry" infla mmatory arthritis of peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a decisive influence on osseous pathology. A total of 38 cas es from a study during the years 1982 to 1992 is analysed with regard to mo rphological characteristics. Rare cases with diaphyseal and pelvic hyperost otic lesions subsequent to bland sclerosing osteomyelitis constitute an ove rlapping region to chronic recurrent multifocal osteomyelitis (CRMO) and il lustrate the relationship between hyperostotic spondarthritis and CRMO. The syndromes of "acquired hyper ostosis" and "SAPHO", the former more radiolo gically oriented and the latter more clinically oriented, together with mai nly CRMO and hyperostotic spondarthritis and its forms, constitute the "Spo nd, hyp, pp.". Although hyperostosis is a guidepost for the radiologist and SAPHO symptomes are one for the clinician, the syndrome does not represent a diagnosis by itself and requires further differentiation. In this report the entity "Spond. hyp. pp." is considered and requires contributions from rheumatologically and osteologically oriented radiologist.