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A Fatal Case of Disseminated Intravascular Coagulation, Gastrointestinal Hemorrhage, and Hemorrhagic Bullous Lesions With Systemic Lupus Erythematosus FREE TO VIEW

Gaja Shaughnessy, MD; Andres Borja Alvarez, MD; John Moss, MD
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Mayo Clinic Florida, Jacksonville, FL

Chest. 2015;148(4_MeetingAbstracts):264A. doi:10.1378/chest.2249045
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SESSION TITLE: Critical Care Student/Resident Case Report Posters I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Systemic lupus erythematosus (SLE) is autoimmune disease with variable symptoms. While dermatologic manifestations and coagulopathies are common in SLE, we encountered a case with a unique initial presentation.

CASE PRESENTATION: 65-year-old female with medical history of diabetes, chronic kidney disease, hypertension treated with hydralazine, and gastric sleeve surgery initially presented with petechial rash, fatigue, and pancytopenia with unremarkable bone marrow. She was later admitted with generalized hemorrhagic bullae, spontaneous spinal intradural hematoma requiring evacuation, acute kidney injury requiring dialysis, and anemia requiring transfusions. An episode of hematemesis prompted intubation. She was transferred to our facility intubated and on corticosteroids due to positive antinuclear antibody (ANA). Physical exam only revealed generalized hemorrhagic blisters and oral ulcers. Laboratory tests showed pancytopenia, activated partial thromboplastin time 68.3s, prothrombin time 20.8s, fibrinogen 131mg/dL, positive fibrin monomers, low coagulation factors, elevated antibodies (ANA, ds-DNA antibodies, anti-histone antibodies, lupus anticoagulant, c-anti-neutrophil cytoplasmic antibody, myeloperoxidase), and low complement. She was diagnosed with disseminated intravascular coagulation (DIC) and SLE. Skin biopsy was negative for vasculitis or specific dermatosis. Her bullae improved with steroids, but she developed diffuse gastrointestinal bleed not amenable to endoscopic therapy, requiring daily transfusions. Plasmapheresis and cyclosporine were initiated, but bleeding persisted and renal function worsened. Her family decided to withdraw life support.

DISCUSSION: DIC in SLE is rare, with one review reporting only 24 cases in the literature. The pathogenesis is not clear and may be related to vasculitis or anti-phospholipid antibodies. Skin involvement occurs in 75%, but bullous lesions are uncommon. Blisters may form due to vacuolar degeneration of epidermal basal layer or antibodies against collagen VII. There have been cases of spinal epidural hemorrhage reported in SLE. This is the first case of spontaneous intradural hematoma in SLE to the best of our knowledge.

CONCLUSIONS: Our case has a very unusual constellation of SLE manifestations. DIC and bullous lesions are rarely seen in SLE and no cases of spontaneous spinal intradural hematoma have been reported.

Reference #1: Obermoser G, Sontheimer RD, Zelger B. Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates. Lupus. 2010;19(9):1050-1070.

Reference #2: Goker B, Block JA. Spinal epidural hematoma complicating active systemic lupus erythematosus. Arthritis Rheum. 1999;42.3: 577-578.

Reference #3: Kageyama Y, Yagi T, Miyairi M. Systemic lupus erythematosus associated with massive ascites and pleural effusion in a patient who presented with disseminated intravascular coagulation. Intern Med. 2002; 41(2):161-166.

DISCLOSURE: The following authors have nothing to disclose: Gaja Shaughnessy, Andres Borja Alvarez, John Moss

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