Steroid Unresponsive Sweetʼs Syndrome in a Patient with Crohnʼs Disease

Abstract

Sweet's Syndrome, or acute febrile neutrophilic dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and tender, erythematous, well-demarcated papules and plaques which show dense neutrophilic infiltrates on histologic examination. Although it may occur in the absence of known disease, Sweet's Syndrome can occur with diseases such as Leukemia, Rheumatoid Arthritis, and Inflammatory Bowel Disease. A 34 year old female presented with an exacerbation of Crohn's Disease and was treated with steroids, fluid rehydration, anti-emetics, pain medication and azathioprine. On day 5 of hospitalization she was noted to have a low grade temperature, moderate leukocytosis and a burning, non-pruritic rash. Scattered vesicles were present on her face, neck, both sides of the upper chest and back, both lower extremities and the proximal phalanx of her left middle finger. Some vesicles had central areas of necrosis and one of her fingers demonstrated a violaceous necrotic plaque. A biopsy revealed a neutrophilic pustular dermatitis and a diagnosis of Sweet's Syndrome was made. Despite treatment with a number of therapies to include colchicine, prednisone, and infliximab, her rash as well as her Crohn's symptoms continued to worsen. Having failed medical therapy for both her Crohn's Disease and Sweet's Syndrome, she ultimately underwent a proctocolectomy, which subsequently brought about complete resolution of her GI symptoms and skin lesions. A literature search failed to reveal a similar case in which a patient with Crohn's Disease and Sweet's Syndrome who failed medical therapy had such a profound response to surgical therapy. Our case prompts the idea that patients with Crohn's Disease and Sweet's Syndrome who do not respond to medical therapy may benefit with regards to overall morbidity with early surgical intervention. Further investigation is warranted.[figure1][figure2]FigureFigure