Rose Geoffrey E, Barnes Eric A, Uddin Jimmy M
Moorfields Eye Hospital, London, England.
Ophthalmology. 2003 Apr;110(4):801-5. doi: 10.1016/S0161-6420(02)01966-8.
To describe the clinical characteristics of periocular pyoderma gangrenosum and to highlight features that may encourage early diagnosis of this extremely rare condition.
Retrospective, noncomparative, interventional case series.
Four patients with periocular pyoderma gangrenosum were treated in the Orbital Unit at Moorfields Eye Hospital over the course of a decade.
Presenting clinical features, therapy, and outcome.
Four patients (female) between the ages of 56 and 75 years (mean, 65 years; median, 64 years) sought treatment for slowly evolving, painful, unilateral blue-gray swellings of the pretarsal tissues of the lower (1 case), upper (1 case), or both eyelids (2 cases). The swelling progressed to frank tissue necrosis and loss of full-thickness eyelid, with patchy sparing of the lid margin or lashes; in some cases, there was a very distinctive preservation of the pretarsal marginal artery across full-thickness eyelid defects. The lid loss characteristically involved the lateral one third of the lower eyelid (3 of 4 lids), the central part of the upper eyelid (3 of 4 lids) and, in one case, extending into the postseptal tissues in the inferotemporal quadrant of the orbit. In 3 patients, the pyoderma, often associated with a positive serum rheumatoid factor (three of three cases where measured), responded well to systemic immunosuppression and eyelid repair was undertaken during the quiescent phase. In a single patient, relapsing disease led to loss of the eye as a result of involvement of the globe and deep orbital tissues.
Although an extremely rare condition, periocular pyoderma gangrenosum has a typical clinical appearance, and early recognition and immunosuppression will reduce the ocular morbidity.
描述眼周坏疽性脓皮病的临床特征，并强调有助于早期诊断这种极其罕见疾病的特征。
回顾性、非对照、干预性病例系列研究。
在十年间，四名患有眼周坏疽性脓皮病的患者在摩尔菲尔德眼科医院眼眶科接受治疗。
呈现的临床特征、治疗方法及结果。
四名患者（均为女性），年龄在56至75岁之间（平均65岁；中位数64岁），因下睑（1例）、上睑（1例）或上下睑（2例）睑前组织缓慢进展、疼痛、单侧蓝灰色肿胀前来就诊。肿胀进展为明显的组织坏死和全层眼睑缺失，睑缘或睫毛有散在保留；在某些情况下，横跨全层眼睑缺损处有一条非常独特的睑前边缘动脉得以保留。眼睑缺失的特征性表现为下睑外侧三分之一（4只眼中的3只）、上睑中央部分（4只眼中的3只）受累，且有1例延伸至眼眶颞下象限的眶隔后组织。在3例患者中，脓皮病常与血清类风湿因子阳性相关（检测的3例均为阳性），对全身免疫抑制治疗反应良好，在病情静止期进行了眼睑修复。在1例患者中，疾病复发导致眼球和深部眼眶组织受累，最终失明。
尽管眼周坏疽性脓皮病极其罕见，但具有典型的临床表现，早期识别和免疫抑制可降低眼部发病率。
