DIFFUSE ALVEOLAR HEMORRHAGE AS A RARE COMPLICATION OF SWEET SYNDROME

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sweet’s syndrome (SS) is a rare inflammatory condition, characterized by fever, neutrophilia and skin lesions in the form of papules, nodules, plaques and rarely bullae. It typically occurs in middle age female, with around 70% of cases being idiopathic. While the disease may involve many organ systems, involvement of pulmonary system is rare (1,2). Systemic corticosteroids is the treatment of choice with favorable clinical outcomes. We present a case of sweet syndrome with pulmonary involvement presenting as diffuse alveolar hemorrhage. CASE PRESENTATION: Our patient is a 62 year old female who presented with Acute Hypoxemic respiratory failure secondary to Diffuse Alveolar Hemorrhage (DAH). An extensive Rheumatologic workup failed to indicate a possible etiology, until the skin biopsy returned positive for an intense neutrophilic infiltration of the epidermal layers of skin, consistent with Sweet Syndrome. A Lung biopsy was also negative for any vasculitis changes, while being suggestive of a diagnosis of Sweet Syndrome. She responded well to intravenous and oral corticosteroids, with full resolution of her respiratory and dermatological lesions. She was maintained on oral Prednisone in the outpatient setting with good clinical response. The patient then returned to our emergency department for new skin lesions. Initial physical exam was significant for purpuric papules on the dorsum of the left hand with ecchymosis and scattered bullae. The rest of the physical exam, laboratory investigations and imaging were within normal limits. The patient then developed hemoptysis. A chest x-ray revealed the development of left sided diffuse patchy opacities. The Patient was eventually intubated for acute hypoxemic respiratory failure. A Bronchoscopy revealed active bleeding from all left segmental bronchi. A skin biopsy showed pathological findings consistent with sweet syndrome (see figure), while being negative for vasculitis. Despite Pulse dose Methylprednisolone 1gram daily, and maximal supportive care , the progressed to develop refractory shock and Multi organ Failure, succumbing to her illness within the next 36 hours DISCUSSION: SS also known as acute febrile neutrophilic dermatosis is a rare inflammatory condition. It typically presents in three clinical settings: idiopathic or classic, malignancy associated and drug-induced sweet syndrome. Classic sweet syndrome commonly affects women between ages of 30 to 60, with no racial predilection. It is usually a multi system disease, with pulmonary involvement being rare, with symptoms ranging from flu like symptoms to acute respiratory distress syndrome. Most of patient responds well to prednisone. CONCLUSIONS: Our case highlights the importance of life threatening early recognition of the pulmonary manifestations in SS, and we emphasize the need for early and aggressive corticosteroid therapy to prevent severe respiratory compromise. Reference #1: Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. Published 2007 Jul 26. https://doi.org/10.1186/1750-1172-2-34 Reference #2: Villarreal-Villarreal CD1, Ocampo-Candiani J1, Villarreal-Martínez A2 Sweet Syndrome: A Review and Update. Actas Dermosifiliogr. 2016 Jun;107(5):369-78. https://doi.org/10.1016/j.ad.2015.12.001. Epub 2016 Jan 2 Reference #3: Vettakkara KMN, Banerjee S, Mittal A, et al. Not so sweet; severe Sweet's syndrome presenting as SIRS and pleural effusion. J Family Med Prim Care. 2018;7(6):1584-1587 DISCLOSURES: No relevant relationships by nargis jilani, source=Web Response No relevant relationships by Ravi Manglani, source=Web Response