SAPHO综合征1例

SAPHO综合征有皮肤病变和骨关节病变.皮肤特征性表现为掌跖脓疱病、脓疱型银屑病、聚合性痤疮等;骨关节病变表现为骨肥厚、硬化和关节炎.本例有掌跖脓疱病,脓疱性银屑病,双侧第一前肋增生肥厚,胸肋关节增生硬化.皮肤病理示银屑病改变,故确诊为SAPHO综合征.     

SAPHO综合征14例临床特征与治疗

目的 探讨SAPHO综合征患者的临床特征。方法 对14例SAPHO综合征患者的临床资料、治疗方案及临床疗效进行回顾性分析。结果 本组14例患者中男8例，女6例，平均发病年龄(28.43±13.38)岁。14例均有皮肤损害(掌跖脓疱病8例，寻常型痤疮2例，囊肿型痤疮3例，聚合型痤疮1例)。影像学中病变累及的骨关节依次为胸前壁(13/14)、骶髂关节(11/14)、四肢骨关节(6/14)、脊柱(6/14)、肩关节(3/14)、骨盆(2/14)。13例患者单用或联合使用非甾体类抗炎药、糖皮质激素、改善病情的抗风湿药和肿瘤坏死因子-α(TNF-α)拮抗剂治疗，其中9例患者先后联合TNF-α拮抗剂治疗。对13例患者进行随访，9例患者单用或联合糖皮质激素、非甾体类抗炎药和DMARDs治疗后皮肤损害或骨关节肿痛症状控制欠佳，其中5例联合TNF-α抑制剂治疗后皮肤和骨关节症状均有不同程度改善，2例患者联合使用英夫利西单抗后皮疹加重后改为益赛普，1例拒绝治疗。结论 本组SAPHO综合征男性多见，主要受累的骨关节是前胸壁，皮肤损害主要表现为掌跖脓疱病，且多见于女性，重度痤疮多见于男性。TNF-α拮抗剂对难...     

5例SAPHO综合征的临床特点

目的:了解SAPHO综合征的临床特点.方法:回顾分析2014年9月至2020年1月香港大学深圳医院皮肤科确诊的5例SAPHO综合征患者的临床资料,包括患者的临床表现、实验室检查、影像学检查、治疗及转归等信息.结果:5例患者均出现掌跖脓疱,其中1例伴急性泛发性发疹性脓疱病、1例伴痤疮、1例受累骨关节局部皮肤红肿.关节表现...     

SAPHO综合征22例分析

目的 探讨SAPHO综合征的临床特点.方法 对22例SAPHO综合征患者的临床资料进行登记并分析.结果 22例中男7例,女15例,皮损出现的平均年龄为45岁,骨关节受累平均年龄为44岁.皮损表现为掌跖脓疱病者21例,暴发性痤疮者1例.骨关节受累中前胸壁受累19例,外周关节受累4例,骶髂关节受累2例.10例患者关节受累早于皮损发生,9例患者皮损早于关节受累,3例患者皮损与关节受累同时出现.关节受累与皮损出现平均间隔2.7年,最长达20年.结论 皮肤科就诊的SAPHO综合征患者以中年女性多见.掌跖脓疱病和前胸壁骨关节受累是最常见的临床表现.     

SAPHO综合征5例并文献复习

目的探讨SAPHO综合征的临床特征,提高临床医生对本病的认识。方法回顾性分析5例SAPHO综合征患者相关资料,并结合文献讨论。结果 5例患者为4男1女,4例皮损表现为掌跖脓疱病,1例表现为聚合性痤疮。其骨关节损害均在皮肤损害之后出现。有4例患者行骨扫描检查,均有病变区的核素浓聚(可见牛头征)。5例均累及胸锁关节,1例累及腕关节、指关节、左侧踝关节。2例胸骨受累,1例足骨受累。结论 SAPHO综合征具有一定的临床和影像学特点,可以为临床诊断提供帮助。     

SAPH0综合征研究进展

SAPHO综合征是一种少见的慢性无菌性炎症性疾病,主要表现为滑膜炎、痤疮、脓疱病、骨肥厚和骨炎.本病具有遗传易感性,并可能与痤疮丙酸杆菌等某些低毒性微生物感染有关.非甾体类抗炎药、抗生素和改善疾病症状的抗风湿药物等传统药物疗效欠佳.近年来用二磷酸盐类钙代谢调节剂和抗肿瘤坏死了因子-α生物制剂治疗某些患者取得较满意疗效.     

无菌性脓疱病研究进展

无菌性脓疱病系非感染性非毛囊性脓疱病,临床表现为脓疱、发热、全身炎症反应。分为泛发型及局限型,泛发型包括全身泛发性脓疱性银屑病（GPP）、急性泛发性发疹性脓疱病（AGEP）、角层下脓疱病（SPD）、自身炎症性脓疱病;局限型包括掌跖脓疱病、连续性肢端皮炎（ACH）、SAPHO综合征（滑膜炎、痤疮、脓疱病、骨肥厚、骨髓炎综合征）、皱褶部位无菌性脓疱病（amicrobial pustulosis of the folds,APF）……     

SAPHO综合征16例临床分析

目的分析SAPHO综合征的临床特征,提高临床医生对本病的认识。方法对16例SAPHO综合征患者的一般资料、临床表现、实验室检查及治疗等进行分析。结果 16例SAPHO综合征患者中,男9例,女7例。掌跖脓疱病14例,重度痤疮2例。胸锁肋关节受累16例,骶髂关节炎10例,僵硬的脊柱骨肥厚4例,周围关节炎11例。C-反应蛋白和血沉轻度升高各9例。以非甾体类抗炎药联合白芍总苷及中药治疗有一定疗效。结论 SAPHO综合征以皮肤病变和骨关节病变为主要临床表现。非甾体类抗炎药、白芍总苷及相关中药可有效缓解骨关节疼痛和皮损。     

以掌趾脓疱病为首发SAPHO综合征的诊治体会

患者女,43岁,掌跖脓疱伴关节疼痛1年余,以脓疱病、胸肋关及腰骶关节疼痛为主要表现。曾被误诊为足癣,经对症治疗效果不佳,后至北京协和医院诊断为SAPHO综合征。来江苏省中医院皮肤科给予抗过敏治疗、光疗以及传统医学等综合疗法,病情有所好转,目前随访中。     

SAPHO综合征研究进展

SAPHO综合征(synovitis-acne-pustulosis-hyperostosis-osteitis syndrome)是指由滑膜炎(synovitis)、痤疮(acne)、脓疱病(pustulosis)、骨肥厚(hyperostosis)和骨炎(osteitis)组成的一组少见的慢性无菌性炎症综合征,临床表现多样化,主要累及皮肤和骨关节,影像学可见特征性骨肥厚及骨炎。近来随着对疾病的认识增加,该病报道逐渐增多但治疗比较困难,生物制剂为SAPHO治疗提供新的选择。     

Stress in a case of SAPHO syndrome

In this article, we describe a case of seemingly stress-induced SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome in a man who exhibited the central features of palmoplantar psoriasis and anterior chest involvement. We also review the etiology, pathogenesis, and treatment of SAPHO syndrome and emphasize the important differences between this syndrome and psoriatic arthritis.     

Long-Term Treatment of Severe SAPHO Syndrome with Adalimumab

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome defines an association of inflammatory cutaneous disorders with osteoarticular manifestations and represents a clinical and therapeutic challenge. We report a case of severe SAPHO syndrome with acne conglobata and a diffuse involvement of the anterior chest wall and sacroiliac joints that required treatment with isotretinoin and adalimumab, a new fully human anti-tumor necrosis factor (TNF)-α monoclonal antibody. Combination treatment determined a complete clinical remission of cutaneous and osteoarticular manifestations after 48 weeks. Despite maintenance of clinical remission, follow-up imaging studies after 24 months of adalimumab monotherapy revealed osteoarticular disease progression, with features of inflammatory osteitis. TNFα antagonists have been used as third-line therapy for SAPHO syndrome in single case reports or case series, but these lack consistent long-term follow-up. SAPHO syndrome can present an intermittent-favorable course in the majority of cases as well as a chronic-progressive course, the latter requiring aggressive combination treatment with TNFα antagonists and conventional systemic agents.     

Pustulotic arthro‐osteitis associated with palmoplantar pustulosis

Palmoplantar pustulosis (PPP) is a chronic inflammatory disorder characterized by sterile pustules predominantly involving the palms and soles of middle‐aged women. PPP frequently develops or exacerbates following focal infections, such as tonsillitis, odontogenic infection and sinusitis, either with or without arthralgia and/or extra‐palmoplantar lesions. Pustulotic arthro‐osteitis (PAO) is a joint comorbidity of PPP, most often affecting the anterior chest wall. PAO is sometimes regarded as the same entity as synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome, and may be a subtype or incomplete type of SAPHO syndrome; however, there are several differences. In Japanese patients, PPP with PAO is frequently seen, whereas SAPHO syndrome in the true meaning is rare. A difference of incidence depending on race suggests that different genetic backgrounds may be responsible for susceptibility to these disorders. Bacterial infection, especially Propionibacterium acnes, is suggested to play an important role in the pathogenesis of SAPHO syndrome. P. acnes is responsible for acne, however, bacterium is unassociated with PPP skin lesions which are characterized by sterile pustules. On the other hand, PAO is frequently triggered by focal infection, and treatment of focal infection results in dramatic effects on the release of joint pain. This paper reviews current insights into the clinicopathophysiology of PAO, and discusses its possible mechanisms in comparison with SAPHO syndrome.     

Do you know this syndrome?

The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) includes a group of findings characterized by bone lesions usually located on the anterior chest wall, often associated with skin lesions. We report the case of a 47 years old patient, with osteochondritis at costoesternal and manubrium-sternal joints, besides of palmar-plantar pustulosis. The diagnosis is predominantly clinical and there are several treatment options described in the literature.     

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with review of the relevant published work

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is characterized by various dermatological manifestations and osteoarthropathy frequently localized to the anterior chest wall. Dermatologists should be familiar with this syndrome. Its early diagnosis is important to avoid prolonged antibiotic treatments and unnecessary invasive procedures. We report a new case of this syndrome with review of the relevant published work.     

SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate: a case report and review of the literature

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare inflammatory condition describing the combination of skin, bone, and joint manifestations that has a heterogeneous presentation. We report a case of severe SAPHO syndrome in association with hidradenitis suppurativa and pyoderma gangrenosum in a 27‐year‐old male. The patient had an initial migratory arthritis affecting the knees, ankles, metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulder, and lower back, which progressed to a persistent arthritis and swelling at the sternum, shoulders, wrists, hands, feet, and lower back. Radiographic changes were consistent with the diagnosis of SAPHO syndrome. Serum proinflammatory cytokine levels were significantly elevated and improved substantially after 3 months of therapy. Rationale for therapy in this patient was the observation that tumor necrosis alpha antagonists have been successfully used in SAPHO syndrome, and since arthropathy was so prominent in our patient, we elected to use adalimumab combined with methotrexate.     

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Associated Neutrophilic Dermatoses: A Report of Seven Cases and Review of the Literature

Abstract:   A growing body of literature has identified the association between neutrophilic dermatoses and multifocal, aseptic bone lesions in children, termed chronic recurrent multifocal osteomyelitis (CRMO). Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy.     

SAPHO syndrome: report of three cases and review of the literature.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is an acronym that describes a seronegative syndrome of bone and joint lesions, frequently localized to the anterior chest wall, associated with variable dermatologic manifestations. Dermatologists should be aware of this entity, as early diagnosis can prevent unnecessary surgery and avoid prolonged ineffective antibiotic therapy. We report three new cases and review the literature.