Does SAPHO syndrome exist in dermatology?

In the late 1960s, palmoplantar pustulosis (PPP) with sternocostoclavicular arthropathy was first described in Japan, predominantly affecting women in the perimenopausal age. In the 1970s, the chronic non-bacterial osteomyelitis and chronic recurrent multifocal osteomyelitis were initially observed in paediatric patients with approximately 70% girls. Acne fulminans accompanied by polyarthralgia have been observed since early 1970s, which almost exclusively occurs in adolescent boys. Report on spondyloarthropathy associated with hidradenitis suppurativa can be traced back to 1982. The SAPHO syndrome was coined in 1987 to lump together synovitis, acne, pustulosis, hyperostosis and osteitis to conceptualize a group of inflammatory osteocutaneous diseases of unclear etiopathogenesis and ill-defined associations spanning disparate age and gender groups. From historical view, Sasaki syndrome is proposed to replace SAPHO syndrome to represent PPP with sternocostoclavicular arthropathy in the absence of other skin manifestations. Hidradenitis suppurativa is folliculitis in pathogenesis and no longer classified as acne. PPP accompanied by psoriasis vulgaris is more likely psoriasis pustulosa palmoplantaris in dermatological aspect, and the associated arthritis is part of psoriatic arthropathy. Pathophysiology of these disorders is incompletely understood. To echo the advancement of high-throughput sequencing, splitting but not lumping of clinical findings would be a better strategy to decipher these multigenic complex inflammatory disorders.

Pustulotic arthro-osteitis: defining the radiologic spectrum of the disease.

Pustulotic arthro-osteitis (PAO) was termed by Sonozaki et al., who discussed the relationship between palmoplantar pustulosis (PPP) and osteoarticular inflammation. Manifestations of PAO are observed in the anterior chest wall, the spine, the pelvis, the sacroiliac joint, and the long bones. Hyperostosis is a radiological feature of PAO; furthermore, anterior chest wall involvement is common. The term SAPHO syndrome (SAPHO being an acronym for synovitis, acne, pustulosis, hypertostosis, and osteitis) has been coined to describe disease that manifests sterile inflammatory bone lesions together with skin eruptions. SAPHO syndrome groups together the following osteo-articular lesions that have been described as separate medical entities: chronic recurrent multifocal osteomyelitis (CRMO), PAO, and arthro-osteitis associated with a follicular occlusive triad. Osseous changes due to psoriasis vulgaris and generalized pustular psoriasis can be radiologically and clinically distinguishable from osseous changes due to PPP, acne, and CRMO as seronegative spondyloarthropathies.

[SAPHO syndrome: clinico-rheumatologic and radiologic differentiation and classification of a patient sample of 86 cases]. / Das SAPHO-Syndrom: Klinisch-rheumatologische und radiologische Differenzierung und Klassifizierung eines Krankengutes von 86 Fällen.

Synovitis (inflammatory arthritis), acne (pustulosa), pustulosis (psoriasis, palmoplantar pustulosis), hyperostosis (acquired), and ostitis (bland osteomyelitis) are symptoms forming the acronym SAPHO, which is a syndrome of nosologic heterogeneity. All entities forming the SAPHO syndrome are connected by a non-obligate dermatoskeletal association with an aseptic pustulous character. 86 cases were analyzed clinically, radiologically and by histology/histopathology. 31 adult patients showed the typical triad of pustulosis palmo-plantaris (psoriatica, PPP), sterno-costo-clavicular hyperostosis (SCCH), and "productive" spondylopathy, which we define as entity I. spondarthritis hyperostotica pustulopsoriatica (Spond.hyp.pp). Twelve adolescent and 13 adult patients showed entity no. II: chronic recurrent multifocal osteomyelitis (CRMO), being characterized by non-purulent osteomyelitis of plasma-cell sclerotic type, potentially being a reactive inflammatory process. 50% of the adult patients with CRMO showed PPP. Differentiation between these two entities is possible by detection of ossifying enthesiopathy in cases of Spond. hyp.pp and primarily chronic osteomyelitis in cases of CRMO. Two more entities or abortive forms of group I and II are III: the inflammatory syndrome of the anterior chest-wall (ACW syndrome) and IV: the more productive form of isolated sterno-costoclavicular hyperostosis (SCCH). Both are connected quite frequently to HLA-B-27-independent forms of spondarthritis and to pustulous dermatosis. More rarely we find osteo-articular symptoms in cases of acne pustulosa, which form group V: acne-associated spondarthritis and CRMO in the case of acne. Adult forms of CRMO with different forms of appearance (lumosacro-iliac hyperostosis with retroperitobeal fibrosis, pelvic type with affection of the hip-joint) are described. The immunologic theory of a "reactive osteomyelitis" potentially triggered by saprophytes is described. The inverse acne triad is brought in a context of skin symptoms. A case of intercurrent postpartum symptoms together with ulcerative colitis is described. Three cases of patients with Crohn's disease are described. Clinical features, radiological findings, and histopathological elements are brought together to determine the connections between the different entities and the possibilities of differentiation. With these elements together with bone-scan, it is often not necessary to obtain a bone specimen. Therapeutical possibilities, especially concerning CRMO, are discussed. "SAPHO syndrome" is more a sign-post on the way to a more subtle diagnosis when it comes to hyperostotic, skin-associated diseases, and it needs interdisciplinary work to clear the situation.

Sindrome Sapho / Sapho syndrome

En este documento se revisa extensamente la literatura con respecto al síndrome SAPHO, con énfasis en los aspectos históricos, criterios de clasificación, las manifestaciones clínicas, hallazgos de laboratorio y tratamiento. Para lograr este objetivo se buscaron las referencias bibliográficas mas significativas del tema en las bases de datos disponibles