A rare case of penile granulomatosis with polyangiitis: case report and multidisciplinary management approach

Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis presenting with penile involvement in approximately 1% of male patients diagnosed with the disease, making genital manifestations exceedingly rare in this context. We aimed to describe here the case of a 34-year-old male patient with a history of GPA presenting to our attention for a genital ulcer that required a coordinated multidisciplinary management. A 34-year-old man with a known history of systemic GPA presented to the Accident and Emergency (A&E) Department with a dorsal penile shaft abscess that progressed into a necrotic ulcer consistent with a localization of GPA (active vasculitis) based on histopathological examination. The patient underwent escharotomy and split-thickness skin grafting (STSG), which failed to take possibly due to local infection with methicillin-resistant Staphylococcus aureus (MRSA) and/or suspected persistent vasculitic activity. Targeted antimicrobial therapy, corticosteroid-based immunosuppression, and hyperbaric oxygen therapy (HBOT) were subsequently implemented, eventually obtaining secondary-intention wound healing. The total hospital stay was 21 weeks. The patient reported a penile curvature of approximately 110° during erection, resulting in the inability to engage in penetrative intercourse. The Visual Analogue Scale (VAS) for the aesthetic and functional outcomes was reported as unsatisfactory (i.e., 2 out of 5). Penile involvement in GPA necessitates a coordinated multidisciplinary approach. Surgical management of GPA-associated large penile skin defects using STSG should be attempted to minimize the hospital stay and to optimize the aesthetic and functional outcomes; however, patients should be informed about a higher likelihood of complications in this setting. The second-intention healing process for large genital skin defects due to GPA may require long hospital stays and may be associated with severe functional issues despite optimal medical management. HBOT may be beneficial in select cases of GPA with genital involvement. Further studies are needed to develop evidence-based guidelines for the management of GPA genital lesions.