Multiple Orocutaneous Extraintestinal Manifestations in Ulcerative Colitis Patient: Complete Response to Ustekinumab

Pyostomatitis vegetans (PV) is characterized by exophyticerythematous pustules with superficial erosions of the oral mucosa, while pyoderma gangrenosum (PG) is a rare skin disorder characterized by the development of painful and deeply ulcerated necrotic areas.1 The diagnosis of PV and PG is based on clinical features while histology is unspecific and not always required.2 We report a case of a 73-year-old woman with steroiddependent, long-standing pancolonic ulcerative colitis (UC) previously treated with azathioprine and naïve to biologics. She was admitted to hospital for sepsis (fever 39 °C, white blood cell count 18 000, C-reactive protein >200 mg/dL, procalcitonin 0.75 ng/mL) and moderate UC relapse (partial Mayo score 10, Mayo endoscopic subscore 1) with multiple painful, ulcerated, vegetative and purulent lesions in the oral cavity and skin (leg and sacral regions) (Figure 1). Intravenous (IV) piperacillin-Tazobactam was started and sepsis resolved after 3 days. After dermatological assessment a diagnosis of multiple PG and oral PV was made, IV methylprednisolone 60 mg/d was started. Remission of intestinal symptoms and improvement of orocutaneous lesions were observed until steroid tapering, when orocutaneous lesions worsened. Due to the age of the patient and the recent septic event, anti-Tumor necrosis factor (TNF) treatment was considered not indicated and IV ustekinumab 390 mg was started followed by a maintenance regimen with 90 mg every 8 weeks. Complete healing of the orocutaneous lesions and clinical and biochemical remission (partial Mayo score 0, C-reactive protein normalization, fecal calprotectin [removed].