A woman in her late 70s with a history of immunoglobulin A monoclonal gammopathy of unknown significance presented with a tender, draining lesion of the central face despite several courses of treatment with antibiotics (Figure, A). The patient lived in Southern California but had visited Kenya for an extended trip a few years prior. Review of systems was negative for fevers, night sweats, weight loss, headache, vision changes, respiratory distress, or arthralgias. On the left nasal dorsum and medial cheek, there was a 3 × 4 cm violaceous plaque with focal purulent drainage. Within several weeks, the lesion rapidly expanded into a vegetative, freely draining plaque covering the cheeks and glabella and approaching the medial canthi (Figure, B). Computed tomography revealed findings consistent with abscess that did not involve the orbits, sinuses, or bone. Chest radiography results were unremarkable. Punch biopsy specimens were obtained for histopathological analysis and tissue cultures (Figure, C). Tissue culture results at 2 weeks remained negative, and a QuantiFERON-TB Gold test result was negative.
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A. Pyoderma gangrenosum
Biopsies demonstrated suppurative and granulomatous dermatitis with an unremarkable epidermis; infectious stain results were negative. An extensive infectious workup, including final tissue cultures/polymerase chain reactions and serology, yielded negative results. Autoimmune markers, including antineutrophil cytoplasmic antibodies, were negative. Ophthalmologic examination was normal. A diagnosis of pyoderma gangrenosum (PG) was made. While the results of an infectious workup were pending, the patient did not respond to high-potency topical or intralesional corticosteroids. She received prednisone, 0.5 mg/kg per day, but developed new crusted scalp plaques and a dusky bulla on the left shin that progressed to an ulcer with undermined borders. A biopsy from the shin showed neutrophil-rich dermatitis with secondary vasculitic changes, further supporting the PG diagnosis. Increasing the dose of prednisone to 1 mg/kg per day arrested further growth and resolved the drainage, followed by a slow taper accomplished using steroid-sparing agents, including infliximab, colchicine, and tacrolimus ointment. At 8 months’ follow-up there was ongoing improvement but substantial residual cribriform scarring on the face (Figure, D).
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Brandon L. Adler, MD, Department of Dermatology, Keck School of Medicine, University of Southern California, 1441 Eastlake Ave, Ezralow Tower, Ste 5301, Los Angeles, CA 90033 (firstname.lastname@example.org).
Published Online: September 7, 2022. doi:10.1001/jamadermatol.2022.3756
Conflict of Interest Disclosures: Dr Adler reported grants from AbbVie and personal fees from Skin Research Institute, LLC outside the submitted work. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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