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特发性坏疽性脓皮病的多模式治疗

Multimodal therapy of idiopathic pyoderma gangrenosum.

作者信息

Sharon Victoria, Burrall Barbara, Patel Forum, He Yong, Konia Thomas, Villalobos Itzel Bustos, Maverakis Emanual

机构信息

University of California, Davis.

出版信息

Dermatol Online J. 2014 Jun 15;20(6):13030/qt5s5397rd.

PMID:24945643
Abstract

A 13-year old girl was admitted to the University of California Davis Medical Center for evaluation and treatment of cutaneous bullae and ulcerations over her lower extremities that were refractory to antibiotic therapy and incision and drainage. Her disease continued to worsen with the appearance of multiple new bullae and the progression of old ones into deep ulcers with undermined borders. Biopsy revealed a neutrophilic dermatosis and diagnostic work-up was negative for infectious or autoimmune etiologies. Given her clinical presentation, biopsy results, and negative work-up, a diagnosis of pyoderma gangrenosum (PG) was made and she was started on immunosuppressive medications. The patient was started on a multidrug regimen of prednisone and cyclosporine but remission was not achieved until the addition of adalimumab. After the inflammatory component of her disease was under control, wound care measures were maximized to promote ulcer healing. Wound care measures included compression and debridement. Upon complete closure of all wounds she was successfully transitioned to mycophenolate mofetil monotherapy for maintenance therapy. This case emphasizes the need for combinational therapy to successfully treat severe cases of PG, which are often refractory to monotherapy with prednisone or cyclosporine. It also highlights the importance of appropriate wound care to achieve complete ulcer healing.

摘要

一名13岁女孩因下肢皮肤大疱和溃疡入住加利福尼亚大学戴维斯分校医学中心,这些症状对抗生素治疗以及切开引流均无效。随着多个新的大疱出现,旧的大疱发展为边界潜行的深部溃疡,她的病情持续恶化。活检显示为中性粒细胞性皮肤病,针对感染性或自身免疫性病因的诊断检查结果为阴性。鉴于她的临床表现、活检结果以及检查结果为阴性,诊断为坏疽性脓皮病(PG),并开始使用免疫抑制药物治疗。患者开始使用泼尼松和环孢素的多药联合方案,但直到加用阿达木单抗后才实现缓解。在其疾病的炎症成分得到控制后,最大限度地采取伤口护理措施以促进溃疡愈合。伤口护理措施包括加压和清创。所有伤口完全愈合后,她成功过渡到霉酚酸酯单药维持治疗。该病例强调了联合治疗对于成功治疗严重PG病例的必要性,这些病例通常对泼尼松或环孢素单药治疗无效。它还突出了适当伤口护理对于实现溃疡完全愈合的重要性。

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