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Pyoderma gangrenosum (PG) is a rare ulcerating inflammatory skin disease diagnosed after exclusion of infection, neoplasia, thrombophilia, and other inflammatory conditions. The incidence is between 3 to 10 patients per million and affects slightly more women than men in the second through fifth decades of life.1 The pathogenesis of PG is multifactorial and involves neutrophilic dysfunction, inflammatory mediators, and a genetic predisposition. Considering the diagnosis of PG for nonhealing ulcers that show a predominance of neutrophils on pathologic analysis is important to prevent unnecessary morbidity.
A woman in her 60s with no significant medical history presented with a nonhealing lesion on the left lower eyelid of more than 2 years’ duration. The lesion began suddenly after the use of a concealer that caused burning on application. She had been treated initially by an oculoplastic surgeon with 3 30-day courses of trimethoprim-sulfamethoxazole. In addition, 3 biopsies were performed, from which she was diagnosed as having a benign keratosis with marked acute and chronic inflammation without evidence of malignancy. Findings of stainings and cultures for bacteria and fungi were negative. She then saw a local dermatologist who tried topical mupirocin treatment without improvement and performed an additional culture that revealed coagulase-negative Staphylococcus. At that point she sought another opinion.
Physical examination at the time of presentation revealed an erythematous edematous plaque with slight surface erosion and a rolled violaceous border on the left lower eyelid (Figure 1). Ectropion was also noted. Bacterial culture and a biopsy were performed. The biopsy demonstrated pseudoepitheliomatous hyperplasia with intraepidermal neutrophilic and eosinophilic pustule formation and mixed dermal inflammation with focal abscess formation. Bacterial culture revealed Staphylococcus epidermidis, and fungal and mycobacterial cultures were negative. The diagnosis of PG was favored. She was started on a dapsone regimen, 100 mg/d, but developed blue lips and jitteriness, and the dapsone treatment was stopped. She was then treated with oral prednisone taper over 4 weeks, starting at 40 mg and decreasing by 10 mg/wk, and topical tacrolimus, 0.1%, ointment twice daily. After a month of this treatment, the lesion decreased in size, and treatment with methotrexate, 10 mg/wk, was started. Over the subsequent month, the lesion healed leaving a residual scar with ectropion of the lower eyelid.
Erythematous plaque with crust and rolled violaceous borders on initial presentation.
She then underwent ectropion repair with grafting from the right clavicle. Immediately thereafter, she developed a painful ulceration at the graft site and was treated by the oculoplastic surgeon with oral trimethoprim-sulfamethoxazole and topical erythromycin ointment.
Examination 9 months later revealed superficial ulcerations with rolled violaceous borders at the site of the donor skin for the graft (Figure 2). Treatment with clobetasol, 0.05%, topical ointment twice daily led to resolution of the ulceration within a month.
Erythematous ulcerated plaques with violaceous rolled borders at ectropion repair graft site.
The pathogenesis of PG is unknown. It occurs in association with inflammatory bowel disease, rheumatologic conditions, and hematologic disorders.1 Ocular involvement in PG is rare but has been reported to affect the eye, orbit, and ocular adnexa with presentations ranging from orbital destruction to sclerokeratitis.2-4 Similar reports of PG causing ectropion have been reported3,5 and highlight the concern for formation of PG lesions at sites of surgical incisions, such as graft sites, coined postoperative PG.6 Pathergy has been described in roughly 20% to 30% of patients with PG,1 and given this risk, preoperative and postoperative immunosuppression with systemic corticosteroids has been recommended. Use of oral prednisone at the time of surgery might have prevented the development of the new ulceration in this patient.
Corresponding Author: Caren Campbell, MD, Division of Dermatology, University of Louisville, 3810 Springhurst Blvd, Ste 200, Louisville, KY 40241 (email@example.com).
Published Online: March 2, 2016. doi:10.1001/jamadermatol.2016.0062.
Conflict of Interest Disclosures: None reported.
Disclaimer: While Dr Callen is Associate Editor of JAMA Dermatology, he had no part in the review of the manuscript or the decision to publish it.
Additional Contributions: We thank the patient for granting permission to publish this information.
Campbell C, Callen JP. Misdiagnosed Periocular Pyoderma Gangrenosum Requiring Ectropion Repair With Development of Second Lesion of Pyoderma Gangrenosum at Graft Site. JAMA Dermatol. 2016;152(4):491–492. doi:10.1001/jamadermatol.2016.0062
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