The New England Journal of MedicineEvolution of Pyoderma Gangrenosum

Pyoderma Gangrenosum and Aseptic Abscess Syndrome: Uncovering Autoinflammatory Connections

A comprehensive examination of a 32-year-old man with recurrent abscesses and a facial skin lesion led to the diagnosis of pyoderma gangrenosum associated with aseptic abscess syndrome, highlighting the complexity of autoinflammatory disorders and their management. The clinical progression, diagnostic process, and therapeutic interventions provide crucial insights into the management of such elusive conditions in clinical practice.

Key Points:

• A 32-year-old male patient presented with a history of recurrent, culture-negative abscesses in multiple organs and a rapidly progressing facial ulcer.
• Initial diagnostic efforts, including microbial cultures and tests for rheumatologic conditions and immunodeficiency, returned negative, complicating the diagnostic process.
• The physical presentation included a skin ulcer with a violaceous border, initially appearing as an umbilicated, tender papule that rapidly enlarged within days.
• Histopathological examination revealed a diffuse neutrophilic infiltrate without any detectable organisms, a characteristic often seen in inflammatory but non-infectious processes.
• Absence of inflammatory bowel disease was confirmed via colonoscopy, ruling out one common associated condition of pyoderma gangrenosum.
• The diagnosis of pyoderma gangrenosum was linked with aseptic abscess syndrome, emphasizing the autoinflammatory nature of the disease.
• Treatment was initiated with colchicine and a tapering course of oral glucocorticoids, leading to noticeable improvement in the skin lesion within one day and significant resolution over two weeks.
• By the 3-month follow-up, both the facial ulceration and systemic abscesses had substantially subsided, indicating effective management of the underlying autoinflammatory disorder.

Pyoderema gangrenosum (PG) is a rare disease with an estimated incidence of 3–10 persons per ten million population. The peak age of incidence is 20–50 years and is more common in women than men.

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