Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with clinical and radiologic presentation in relation to the SAPHO syndrome

Abstract

Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp-Schilling), corresponding nosologically to pustulotic arthroosteitis, is a dermato-skeletal "double system" disease of adults. It consists of the triad (a) palmo-plantar pustulosis (Ppp) or, alternatively, Königsbeck-Barber-type psoriasis, (b) sternocostoclavicular hyperostosis (SCCH), and (c) truncal-skeletal changes with syndesmophite-like, hyperostotic and/or parasyndesmophite-like 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" inflammatory arthritis of peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a decisive influence on osseous pathology. A total of 38 cases from a study during the years 1982 to 1992 is analysed with regard to morphological characteristics. Rare cases with diaphyseal and pelvic hyperostotic lesions subsequent to bland sclerosing osteomyelitis constitute an overlapping region to chronic recurrent multifocal osteomyelitis (CRMO) and illustrate the relationship between hyperostotic spondarthritis and CRMO. The syndromes of "acquired hyperostosis" and "SAPHO", the former more radiologically oriented and the latter more clinically oriented, together with mainly CRMO and hyperostotic spondarthritis and its forms, constitute the "Spond.hyp.pp.". Although hyperostosis is a guidepost for the radiologist and SAPHO symptoms 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 required contributions from rheumatologically and osteologically oriented radiologist.