1263

Abstract

Introduction: Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a rare inflammatory disorder in children that presents with cutaneous lesions with dermal neutrophilic infiltrate, fever, and leukocytosis. Extracutaneous manifestations usually include ocular, musculoskeletal, oral, and central nervous system. We present a case of Sweet syndrome in a child presenting with respiratory failure secondary to severe airway involvement. A 9-year-old male with a history of pyoderma gangrenosum, seizure disorder, and Factor V Leiden deficiency presented to a local hospital with several weeks of developing cutaneous rash, ocular discharge, oral ulcers, and dysphagia. He rapidly progressed to respiratory failure requiring nasal intubation under fiberoptic guidance. During intubation, he was noted to have an edematous, friable trachea with mucosal hypertrophy and sloughing. No other airway abnormalities were noted. He had mild leukocytosis with WBC 13.5 and ANC 11. On Hospital Day #4, a repeat bronchoscopy was performed to assess the progression of disease. This revealed diffuse lower airway edema, papillary lesions, epithelial sloughing and copious secretions. Evaluation of lavage fluid obtained from the bronchoscopy showed neutrophilic predominance (89%). Given his history of pyoderma gangrenosum and presenting cutaneous rash, dermatology was also consulted and a skin biopsy that was performed revealed dense neutrophilic dermal infiltrate. Methylprednisolone 2 mg/kg/day was initiated, resulting in a demonstrable improvement in his cutaneous and mucosal lesions, pulmonary compliance and quantity of tracheal secretions. Follow up bronchoscopy prior to extubation revealed mild improvement in airway edema and sloughing and, incidentally, erosion of approximately 60% of his nasal septum with clean mucosal edges and no active signs of necrosis was noted. After several days of intravenous steroid therapy the patient was successfully extubated. Dapsone therapy was initiated upon extubation. The remaining extensive work-up for underlying etiology of Sweet syndrome remains negative. Discussion: Sweet syndrome has been described infrequently, with only several hundred case reports in the literature, and fewer than 50 reports in children and adolescents. This patient met all of the major and minor criteria for Sweet syndrome as described by von den Driesch. While there have been descriptions of mucosal and airway involvement in Sweet syndrome, the extent of airway involvement in this presentation of Sweet syndrome-including nasal septum destruction-is abnormal, especially in a pediatric patient. This case highlights the importance of utilizing a thorough history and physical in uncovering the unusual underlying etiology of acute respiratory failure of our pediatric patient. The prior history of pyoderma gangrenosum combined with the findings during the current admission led to a diagnosis of Sweet syndrome and appropriate therapy.