Diagnosis: Bullous pyoderma gangrenosum
Antibiotic therapy was changed to imipenem and clindamycin intravenously and concomitant high-dose systemic steroid therapy was initiated. Under this treatment regimen, the patient’s clinical state improved rapidly. All microbial cultures and eubacterial polymerase chain reaction (PCR) remained negative.
Five days after escalating antibiotic therapy it was discontinued, while sustaining systemic high-dose steroid therapy. The patient’s condition remained stable, and wound conditions improved. Subsequently, surgical reconstruction of the affected knee joint and tissue defect was performed over several months. As part of a continuous steroid reduction regimen for systemic immunosuppression, tumour necrosis factor (TNF)-alpha inhibitors were introduced. The patient tolerated all subsequent surgical interventions well. Immunosuppressive therapy was eventually able to be stopped after 8 months.
Pyoderma gangrenosum (PG) is a rare dermatological condition with most therapy recommendations based on clinical experience and case reports/series. It is mostly a clinical diagnosis supported by histological findings of deep dermal neutrophilic infiltrate (1, 2). Atypical (bullous) pyoderma gangrenosum (APG) is an even rarer sub-variant (1–3). Often initially misdiagnosed as an infectious process, it presents with bullous and ulcerative skin changes, sometimes with signs of necrosis (4–6). Diagnosis is made by excluding other disease entities. Generally, surgical interventions in this condition pose a risk of recurrence, leading to the pathergy phenomenon. PG has been known to manifest for the first time after surgical interventions, particularly in breast and orthopaedic surgery (5–8). In this context diagnosis is often delayed by mimicking postoperative wound infections, posing a peculiar challenge in postoperative care and management (5, 7–10).
Atypical pyoderma gangrenosum should be considered when anti-infective therapy yields insufficient improvement and dermatological evaluation should be sought. Perioperative management can be challenging and requires interdisciplinary cooperation. Often long-term systemic immunosuppression is necessary to achieve remission.
ACKNOWLEDGEMENT
Patient informed written consent was obtained regarding the anonymous publication of their medical information and images.
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