INTRODUCTION:Sweet’s Syndrome (SS) or acute febrile neutrophilic dermatosis is a disorder characterized by fever, tender erythematous skin lesions formed by dense neutrophilic dermal infiltration, and peripheral neutrophilic leukocytosis. Although this disorder typically involves multiple organ systems, pulmonary involvement is quite rare. We report a case of pulmonary SS in a young woman presenting with acute respiratory failure.
CASE PRESENTATION:A 25 year old previously healthy female first noted several 3-5 mm tender skin lesions on her upper thigh 6 days prior to evaluation. She subsequently developed migratory arthralgias involving the hands, wrists, and ankles. On the day prior to evaluation, her skin lesions progressed to involve both lower extremities, and she developed fever, ankle cellulitis, and severe lower extremity myalgias, prompting an ED evaluation. Her past medical history and family history are unremarkable. She denied medication use. Physical exam revealed a woman in no acute distress, febrile to 101.8 with a lower extremity rash characterized by multiple edematous, violaceous lesions, 10 mm in greatest dimension, and in various stages of healing. Some lesions had central umbilication and yellow, crusting borders (Figure 1). She was admitted and received empiric antibiotics for cellulitis. Over the next 24 hours, she developed progressive hypoxemia. Chest roentgenogram revealed bilateral pulmonary nodules that spread rapidly over the next 48 hours, despite broadened antimicrobial coverage that included Trimethoprim/Sulfa and Amphotericin (Figure 2). She underwent bronchoscopy with BAL and transbronchial biopsy as well as skin biopsy. BAL revealed neutrophilia but no organisms. Transbronchial biopsy showed focal acute and organizing pneumonia. Histopathological examination of her skin biopsy revealed a neutrophilic infiltrate in the mid-dermis. The lung and skin biopsy findings, together with the clinical scenario, met the diagnostic criteria for classical (idiopathic) SS. As SS is commonly associated with malignancy, she was evaluated for both hematogenous and solid organ neoplasms, but none were found. Studies for associated rheumatologic disorders were negative, as were serum markers for vasculitis. Antibiotics were discontinued and she was treated with high-dose corticosteroids (1 gm Solumedrol). Within 12 hours corticosteroid therapy, she had rapid symptom and radiographic resolution. She received a tapering 4-month course of steroids and low dose dapsone. Since discontinuation of therapy, there has been no disease recurrence.
DISCUSSIONS:Sweet’s syndrome, a rare disorder with case reports numbered in the hundreds, is characterized by fever, neutrophilic dermatitis, and a peripheral neutrophilia. Most commonly seen in women between the ages of 30 to 50, SS may occur in both genders at all ages. First reported in 1964 by Robert Sweet, this syndrome has now been associated with malignancy, underlying systemic inflammatory diseases, upper respiratory infection prodromes, drugs, and pregnancy. However 20% of cases are idiopathic. When pulmonary involvement is present, it usually manifests as a dry cough and dyspnea. Chest roentgenograms have widely variable appearances. As in our case of pulmonary SS, the differential diagnosis includes most systemic diseases with mucocutaneous findings such as fungal disease, endocarditis, neoplasm, cellulitis, and viral illness. The diagnosis is typically made by skin biopsy in the appropriate clinical context with both major (abrupt onset of painful skin lesions associated with a neutrophilic infiltrate) and minor criteria (fever, identification of underlying associated illness, rapid response to immunosuppression, or abnormal lab values such as leukocytosis). Upon diagnosis of SS, a thorough evaluation for associated disorders is critical, as treatment is immunosuppressive therapy.
CONCLUSION:Pulmonary Sweet’s Syndrome is an uncommon clinical manifestation of a rare disorder. Diagnosis requires a high index of suspicion and skin biopsy.
DISCLOSURE:Jennifer Fulton, No Financial Disclosure Information; No Product/Research Disclosure Information