SWEET'S SYNDROME IN PATIENTS WITH ULCERATIVE COLITIS: DIAGNOSTIC AND THERAPEUTIC CHALLENGES

Case Presentation A 60-year-old male patient with a history of Ulcerative Colitis (UC) diagnosed in 2020, on Infliximab and Azathioprine with partial control, continuing to experience occasional diarrhea and hematochezia, was admitted to the Emergency Room with painful erythematous plaques on the trunk and upper limbs, associated with palpebral hyperemia and fever. Laboratory results revealed leukocytosis with neutrophilia. In collaboration with Dermatology, a skin biopsy was performed, showing a dense neutrophilic infiltrate in the upper dermis without evidence of vasculitis, with histopathology confirming Sweet's Syndrome (SS). Treatment with Prednisone at 1 mg/kg/day was started, and adjustments were made to the immunosuppressive therapy. The patient showed a rapid clinical response, with complete regression of the lesions and systemic symptoms. The corticosteroid dose was gradually reduced, and the patient remained in remission from the skin lesions.

Discussion Sweet's Syndrome (SS) is a rare, recurrent cutaneous disease with an incidence peak between the fourth and seventh decades of life, predominantly affecting females (4:1). Described by Robert Douglas Sweet in 1964, it is characterized by the sudden appearance of multiple erythematous-violet, elevated, and painful plaques, associated with fever, leukocytosis with neutrophilia, and a dermal neutrophilic infiltrate, often located on the upper parts of the trunk. It is divided into 3 classes: associated with neoplasms, drug-induced, or the classic (idiopathic) form, the latter associated with Inflammatory Bowel Diseases (IBD). Diagnosis is based on the criteria of Su and Liu (1986), defined by the presence of major criteria and at least 2 minor criteria (see table). The cause and pathogenesis remain unknown, but histopathological findings suggest a hypersensitivity reaction related to the increase of pro-inflammatory cytokines, also found in IBDs, such as IL-1, IL-3, IL-6, IL-8, interferon gamma, and colony-stimulating factors, which play an important role in neutrophil activation and migration to the skin, resulting in the characteristic infiltrate. First-line treatment is corticosteroids in pulse therapy, with an initial dose of Prednisone 30-60 mg/day with gradual tapering, in combination with topical treatment. As alternatives, Colchicine and Potassium Iodide can be used.

Conclusion Early recognition, appropriate management, with clinical discussion, and a multidisciplinary approach in collaboration with Dermatology were essential for the positive outcome, highlighting the importance of multidisciplinary care in treating UC with dermatocutaneous manifestations.

Publication History

Article published online:
25 April 2025