SAPHO综合征

SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics.

An entity initially known as chronic recurrent multifocal osteomyelitis(CRMO) was first described in 1972. Subsequently, in 1978, several cases of CRMO were associated with blisters on the palms and soles (palmoplantar pustulosis). Since then, a number of associations between skin conditions and osteoarticular disorders have been reported under a variety of names, including sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, and acne-associated spondyloarthropathy. The term SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.

Radiologic findings

Anterior chest wall (most common site, 65–90% of patients):Hyperostosis, sclerosis and bone hypertrophy especially involving the sternoclavicular joint, often with a soft tissue component.

Spine (33% of patients):Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement.

Long bones (30% of patients):usually metadiaphyseal and located in the distal femur and proximal tibia. It looks like chronic osteomyelitis but will not have a sequestrum or abscess.

Flat bones (10% of patients):mandible and ilium.

Peripheral arthritis has been reported in 92% of cases of SAPHO as well.[citation needed]

In children, the SAPHO syndrome is most likely to affect the metaphysis of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.

SAPHO综合征是主要累及皮肤、骨和关节的一种慢性疾病。SAPHO为下列5个英文单词的缩写，即：滑膜炎（synovitis）、痤疮（acne）、脓疱病（pustulosis）、骨肥厚（hyperostosis）和骨髓炎（osteomyelitis）。

1987年Charnot等经过归纳，取每一病变的首字母，提出SAPHO综合征的命名，并把本综合征的骨关节病变分为与痤疮有联系的风湿性表现（团块、暴发的、汗腺炎、化脓的），掌跖脓疱病，胸锁骨肥厚和慢性复发性骨髓炎四组。所有病人均累及胸锁关节，并引起无菌性骨肥厚性骨炎。

1987年法国医师Charmot等提议将掌跖脓疱病、化脓性汗腺炎或重症痤疮伴发胸肋锁骨肥厚、慢性复发性多灶性关节炎、骶髂关节炎等损害统称为SAPHO综合征。其共同特点是皮肤损害在某一时期均有无菌性脓疱，骨损害包括无菌性骨炎、无菌性骨髓炎和关节炎。