SWEET SYNDROME WITH PULMONARY INVOLVEMENT

Abstract

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sweet syndrome (acute neutrophilic dermatosis) typically presents with cutaneous lesions, fever, and arthralgia. Sweet syndrome with pulmonary involvement is rare and to our knowledge, only 40 cases have been reported in the literature. In this case, we will highlight the clinical and radiographic findings seen in Sweet syndrome with pulmonary involvement. CASE PRESENTATION: A 58-year-old male with past medical history of GERD presented to the ED with worsening SOB and a nonproductive cough. He endorsed recent completion of Levofloxacin therapy for presumed pneumonia. Vital signs were notable for fever of 39º C. Patient was tachypneic and required 3L NC for O2 saturations > 92%. PE revealed decreased LLL sounds as well as erythematous nodules noted on bilateral lower extremities. CBC showed elevated WBC of 18.2. CMP and urinalysis were within normal limits. CT Chest showed bilateral ground glass opacities and minimal pleural effusions. IV Vancomycin and Zosyn were initiated for community acquired pneumonia coverage, however patient continued to spike fevers. Blood and urine cultures remained negative. On day eight of admission, bronchoscopy was performed. BAL and transbronchial biopsy showed extensive neutrophilic infiltrate. Skin biopsy also showed a dense dermal infiltrate composed of neutrophils. Four days after the biopsy, patient developed acute hypoxic respiratory failure requiring ICU admission for intubation. CT Chest showed worsening bilateral pleural effusions and ground glass opacities. Intravenous methylprednisolone was started with rapid improvement in patient's respiratory and cutaneous symptoms. Patient was eventually transitioned to oral prednisone. DISCUSSION: In this case, we present a patient with histopathological and radiographic findings of Sweet syndrome with pulmonary involvement. Our patient fulfills two major and at least two minor criteria of Sweet syndrome including abrupt onset of tender erythematous nodules and a dense dermal neutrophilic infiltrate without leukocytoclastic vasculitis on histology. Minor criteria included fever > 38º C, elevated ESR, neutrophilia, and a dramatic response to treatment with steroids. Prompt transbronchial and skin biopsies in combination with an excellent response to corticosteroid therapy confirmed the diagnosis of Sweet syndrome with pulmonary involvement. Our patient continues to require daily steroid therapy to prevent recurrence of symptoms. CONCLUSIONS: Overall, the aim of this case is to recognize the clinical and radiographic findings of pulmonary Sweet syndrome and to confirm the diagnosis based on the clinical criteria presented. Early initiation of corticosteroid therapy can result in favorable patient outcomes as discussed in this case. REFERENCE #1: Robbins CM, Mason SE, Hughey LC. Sweet Syndrome With Pulmonary Involvement in a Healthy Young Woman. Arch Dermatol. 2009;145(3):344–346. doi:10.1001/archdermatol.2008.610 REFERENCE #2: Fernandez-Bussy S, Labarca G, Cabello F, Cabello H, Folch E, Majid A. Sweet's syndrome with pulmonary involvement: Case report and literature review. Respir Med Case Rep. 2012;6:16-19. Published 2012 Oct 17. doi:10.1016/j.rmcr.2012.08.004 REFERENCE #3: Nofal A, Abdelmaksoud A, Amer H, Nofal E, Yosef A, Gharib K, Albalat W, Eldesouky F, Ebrahim HM, Abdelshafy AS, Fayed H. Sweet's syndrome: diagnostic criteria revisited. J Dtsch Dermatol Ges. 2017 Nov;15(11):1081-1088. doi: 10.1111/ddg.13350. Epub 2017 Oct 5. PMID: 28981179. DISCLOSURES: No relevant relationships by Huma Khan, source=Web Response