33313 Bullous and subcutaneous Sweet syndrome with extracutaneous manifestations: A case report and review of the literature

Abstract

An 18-year-old Caucasian female with no past medical history presented with tense bulla with hemorrhagic and purulent drainage at the bilateral feet, tender and indurated pink nodules on her right ventral forearm, and associated edema of the extremities. She endorsed intermittent fevers, abdominal pain, hematochezia, and arthralgias. Punch biopsy revealed epidermal, dermal, and subcutaneous mixed perivascular and interstitial inflammation with neutrophils. Laboratory studies exhibited leukocytosis with neutrophilic predominance, microcytic anemia, and elevated inflammatory markers. Imaging demonstrated a left sided pleural effusion and splenic abscesses on CT Chest/Abdomen/Pelvis, and enhancements consistent with osteomyelitis, tenosynovitis, and pyomyositis on MRI of the bilateral feet. Colonoscopy performed for a history of hematochezia was consistent with Crohn’s colitis. The patient was diagnosed with bullous and subcutaneous Sweet syndrome with extracutaneous manifestations. She was treated with systemic and topical steroids and transitioned to adalimumab with marked improvement of her condition. Sweet syndrome can have a variety of presentations, but the bullous and subcutaneous variants are considered rarer, typically occurring in the context of hematologic malignancy. Extracutaneous manifestations of Sweet syndrome have been reported including sterile neutrophilic involvement of bone, eyes, kidneys, liver, lungs, muscles, and spleen. Our case demonstrates an atypical presentation of Sweet syndrome with extensive extracutaneous manifestations triggered by Crohn’s colitis, which required a multidisciplinary approach with gastroenterology, rheumatology, podiatry, physical therapy, pulmonology, and infectious disease to appropriately manage. Dermatologists should be aware of the various cutaneous and extracutaneous manifestations of Sweet syndrome in order to facilitate diagnosis and a multidisciplinary treatment approach.