A Case of Acquired Factor V Inhibitor During Bullous Pemphigoid Treatment

Acquired factor V inhibitor (AFVI) is a rare coagulation disorder caused by the production of anti-factor V antibodies in response to infection, surgery, malignancy, autoimmune disease, antibiotics, or other drugs. Its clinical manifestations vary from asymptomatic to severe; hence, optimal treatmen...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2024-09, Vol.16 (9), p.e69090
Main Authors: Ou, Kaho, Arakawa, Haruka, Togashi, Yui, Fujita, Hiroyuki, Matsukura, Setsuko
Format: Article
Language:English
Subjects:
Allergy/Immunology
Dermatology
Hematology
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Summary:Acquired factor V inhibitor (AFVI) is a rare coagulation disorder caused by the production of anti-factor V antibodies in response to infection, surgery, malignancy, autoimmune disease, antibiotics, or other drugs. Its clinical manifestations vary from asymptomatic to severe; hence, optimal treatment is difficult. Bullous pemphigoid (BP) is an autoimmune disorder caused by autoantibodies against dermal-epidermal junction structural proteins. We describe a patient with BP and AFVI, successfully treated with prednisolone. A 78-year-old Japanese man presented with tense hemorrhagic blisters on his trunk and extremities. Owing to his urinary tract infection and advanced age, oral prednisolone was initiated at 20 mg (0.3 mg/kg/day) for BP. Three weeks after treatment, upper gastrointestinal bleeding, hemorrhagic shock, aspiration pneumonia, and hematuria occurred. An elevated anti-BP180 antibody titer (2050 U/mL) indicated BP, and a prolonged activated partial thromboplastin time (aPTT, >180 seconds) indicated a coagulation disorder; the international normalized ratio was too prolonged to be calculated. Based on low factor V activity (
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.69090