Pyoderma Gangrenosum Progression with Angio-Invasive Fungus
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Pyoderma gangrenosum is a neutrophilic dermatosis characterized by painful ulcers that display pathergy . The classic variant of PG usually starts as a small painful papule that can be mistaken for an insect bite and often occurs on the legs. The lesion widens, deepens, and becomes an ulcer with a marked overhanging grey or violaceous edge. Diagnosis of PG can be challenging and requires exclusion of other ulcerative pathologies. Biopsy is advised but may fail to show the distinctive neutrophilic infiltration that would raise suspicion for this diagnosis. Although PG is a poorly understood disorder, most patients recover when properly treated. Current therapy consists of a combination of local wound care, antiinflammatory drugs, antibacterial agents, and immunosuppressants.

A 64-year-old woman, with a history of hypertension, diabetes mellitus and vitiligo, presented for management of biopsy-proven PG that had been present for over a year. At the time of presentation she had a single large ulcer with violaceous border on her left lower leg. Work up for malignancy and other auto-immune diseases was negative.

She presented without any systemic complaints. Her PG was refractory to several standard treatments over the course of a year, including prednisone, cyclosporine, and doxycycline. She also failed adalimumab but had showed response to infliximab infusions when combined with cyclosporine. Topically she was treated with vinegar soaks, tacrolimus cream, and fluocinonide ointment. She had daily dressing changes and care by a wound care specialist. Her ulcer subsequently improved. However, her improvement plateaued at 10 months and the lesion appeared to be progressing.

To stop any further disease progression, which may be from loss of response secondary to neutralizing antibodies to infliximab, she was switched to anakinra, 100mg daily. Her dose of cyclosporine was continued with plans to stop if the lesion improved. She was continued on the topical regimen outlined above. Initially, she had improvement on her one-month follow-up and anakinra was continued along with the rest of her regimen. However, two months after starting anakinra, she reported pain in her leg and felt the lesion was progressing again.

During her follow-up visit, her ulcer had developed a golden-yellow eschar with a black necrotic perimeter. Due to concern for super-imposed infection, a biopsy was collected from this necrotic area, which demonstrated angio-invasive fungus (AIF) and was later cultured to be several mucor species. She was promptly started on anti-fungal treatment with posaconazole and received debridement of the wound. However, due to the extensive progression of the infected ulcer, she received a below-the-knee amputation. She was treated with a wound vacuum and posaconazole post-operatively and has not had recurrence of her PG after amputation.