Pyoderma gangrenosum of the ocular adnexa: a rare condition with characteristic clinical appearances.
OBJECTIVE
To describe the clinical characteristics of periocular pyoderma gangrenosum and to highlight features that may encourage early diagnosis of this extremely rare condition.


METHODS
Retrospective, noncomparative, interventional case series.


METHODS
Four patients with periocular pyoderma gangrenosum were treated in the Orbital Unit at Moorfields Eye Hospital over the course of a decade.


METHODS
Presenting clinical features, therapy, and outcome.


RESULTS
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.


CONCLUSIONS
Although an extremely rare condition, periocular pyoderma gangrenosum has a typical clinical appearance, and early recognition and immunosuppression will reduce the ocular morbidity.
Rose
Geoffrey E
GE
Moorfields Eye Hospital, London, England.
Barnes
Eric A
EA
Uddin
Jimmy M
JM
D000368:Aged; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004847:Epithelial Cells / Q000637:transplantation; D005141:Eyelid Diseases / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007262:Infusions, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D017511:Pyoderma Gangrenosum / Q000175:diagnosis* / Q000188:drug therapy; D019651:Plastic Surgery Procedures; D012189:Retrospective Studies; D012590:Sclera / Q000637:transplantation; D015423:Scleritis / Q000175:diagnosis / Q000188:drug therapy; D033581:Stem Cell Transplantation
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone