Resident Physician UMass Medical School worcester, Massachusetts, United States
Background: Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is a rare inflammatory skin condition characterized by the sudden onset of painful, erythematous papules and plaques. It involves an overproduction of neutrophils and has been linked to systemic diseases, including ulcerative colitis (UC). SS typically presents with tender, erythematous papules that may evolve into plaques, pustules, and nodules, often affecting the face, neck, and other areas, accompanied by fever and neutrophilia. Diagnosis is based on clinical and histological examination. Differentiating SS from similar dermatological conditions like erythema nodosum (EN) and pyoderma gangrenosum is crucial, as treatment strategies differ. Systemic corticosteroids are the first-line treatment for SS but are generally avoided in EN unless severe. This case report emphasizes the timely diagnosis of cutaneous pathologies associated with inflammatory bowel disease (IBD).
Case: A 24-year-old male with recently diagnosed UC presented to the ER with a week of eruptive lesions on his scalp, face, neck, thorax, and extremities. He had biopsy-proven ulcerative pancolitis and completed induction with prednisone and infliximab one month prior to symptom onset. He had initiated methotrexate 12.5 mg one day prior to symptom onset. The painful, papular-pustular lesions rapidly increased in size and number, later ulcerating with purulent drainage. A broad infectious workup was negative, but a punch biopsy on day 2 showed interstitial granulomatous dermatitis with neutrophils, thus meeting the two major criteria for diagnosis. Additionally, three minor criteria were met: fever, history of IBD, and excellent response to steroids. The patient showed dramatic improvement within 24 hours of starting prednisone 1 mg/kg and was later switched to ruxolitinib 5 mg twice daily.
Conclusion: Sweet syndrome is often associated with underlying systemic conditions like IBD. This case highlights the importance of considering SS in UC patients with unusual skin manifestations, especially when there is concern for bacterial infection or in males, where the disease is less common. A timely biopsy can reduce delays in initiating corticosteroids, the first-line treatment for both UC and SS, and decrease unnecessary broad-spectrum antibiotic use. This patient's case highlights the diverse clinical presentations of SS, including large, black and red pyoderma-like ulcers and hemorrhagic papules and plaques while on infliximab. His condition improved with prednisone and ruxolitinib. Given the lack of standardized SS management guidelines, early recognition and appropriate treatment are paramount. This case emphasizes the necessity of a thorough diagnostic approach in UC patients with persistent or atypical cutaneous and systemic symptoms to avoid misdiagnosis and ensure optimal management.
