SHM Converge Scientific Abstract ePoster Gallery-Hematologic Havoc: Severe Thrombocytopenia Caused by Bullous Sle

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This case describes a rare presentation of bullous systemic lupus erythematosus (SLE) in a 22-year-old woman hospitalized with fever, altered mental status, petechiae, and profound thrombocytopenia. She had a history of immune thrombocytopenia and presented with platelet count 6,000/mL, hemoglobin 4.5 g/dL, schistocytes, low haptoglobin, elevated LDH, positive IgG direct Coombs test, and decreased ADAMTS13, initially raising concern for thrombotic thrombocytopenic purpura (TTP) versus Evans syndrome.<br /><br />Three days into admission, she developed a dense bullous rash on her upper extremities. Further workup showed positive ANA and anti-dsDNA antibodies, and punch biopsy confirmed bullous SLE. She was treated with plasmapheresis, rituximab for three weeks, high-dose prednisone, then transitioned to hydroxychloroquine and mycophenolate mofetil.<br /><br />The report emphasizes that bullous SLE is very rare, occurring in less than 1% of SLE cases, but can present with complications including severe thrombocytopenia. In patients with SLE, severe thrombocytopenia may result from TTP, Evans syndrome, or uncontrolled lupus itself. A positive direct Coombs test is unusual in TTP and, combined with prior ITP, supports Evans syndrome. Correctly identifying the cause of thrombocytopenia is important to guide long-term treatment and prevent recurrence.<br /><br />Overall, the case highlights bullous SLE as an uncommon initial manifestation of lupus and underscores the need to consider multiple autoimmune causes when evaluating severe thrombocytopenia in SLE patients.

Asset Subtitle

Cole J. White

Meta Tag

Author List Cole J. White, Kim Jordan, Lauren Spaeth, Michael A. Wiley

Category Clinical Vignettes

Concept Lupus

Concept Systemic Lupus Erythematosus

Concept Thrombocytopenia

Concept Evans Syndrome

Concept Thrombotic Thrombocytopenic Purpura

Distinguished Non-Finalist

Presenter Organization OhioHealth Riverside Methodist Hospital Internal Medicine Residency Program

Presenting Author Cole J. White

Track Adult

Keywords

bullous systemic lupus erythematosus

SLE

thrombocytopenia

thrombotic thrombocytopenic purpura

Evans syndrome

direct Coombs test

anti-dsDNA antibodies

plasmapheresis

rituximab

autoimmune blistering rash

Lupus

Systemic Lupus Erythematosus

Thrombocytopenia

Evans Syndrome

Thrombotic Thrombocytopenic Purpura