Hu SY, Li MC, Hao ZJ, Chai XY, Li PS, Liu Y, Liu LX, Xu Y, Yang PP, Li LE. Bullous pemphigoid associated with acquired hemophilia A: A case report. World J Clin Cases 2025; 13(4): 94294 [PMID: 39917578 DOI: 10.12998/wjcc.v13.i4.94294]
Corresponding Author of This Article
Ling-E Li, BSc, Chief Physician, Department of Dermatology, Shijiazhuang TCM Hospital, No. 233 Zhongshan West Road, Qiaoxi District, Shijiazhuang 050051, Hebei Province, China. zyypfk2008@126.com
Research Domain of This Article
Dermatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Feb 6, 2025; 13(4): 94294 Published online Feb 6, 2025. doi: 10.12998/wjcc.v13.i4.94294
Bullous pemphigoid associated with acquired hemophilia A: A case report
Su-Ye Hu, Meng-Can Li, Zi-Jia Hao, Xu-Ya Chai, Pei-Sai Li, Yang Liu, Li-Xia Liu, Ying Xu, Pan-Pan Yang, Ling-E Li
Su-Ye Hu, Zi-Jia Hao, Xu-Ya Chai, Pei-Sai Li, Yang Liu, Pan-Pan Yang, Ling-E Li, Department of Dermatology, Shijiazhuang Traditional Chinese Medicine Hospital, Shijiazhuang 050051, Hebei Province, China
Meng-Can Li, Graduate School, Hebei University of Traditional Chinese Medicine, Shijiazhuang 050051, Hebei Province, China
Li-Xia Liu, Ying Xu, Department of Pathology, Shijiazhuang Traditional Chinese Medicine Hospital, Shijiazhuang 050051, Hebei Province, China
Co-first authors: Su-Ye Hu and Meng-Can Li.
Co-corresponding authors: Pan-Pan Yang and Ling-E Li.
Author contributions: Chai XY and Hao ZJ performed laboratory testing and clinical data collection; Li PS and Liu Y performed pathological studies; Liu LX and Xu Y approved the pathological studies; Hu SY and Li MC drafted the manuscript; Yang PP and Li LE critically revised the manuscript for important intellectual content. All authors have read and approved the final version.
Supported by Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine, No. 2025313 and No. 2025448.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this case report.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ling-E Li, BSc, Chief Physician, Department of Dermatology, Shijiazhuang TCM Hospital, No. 233 Zhongshan West Road, Qiaoxi District, Shijiazhuang 050051, Hebei Province, China. zyypfk2008@126.com
Received: March 21, 2024 Revised: September 29, 2024 Accepted: November 4, 2024 Published online: February 6, 2025 Processing time: 238 Days and 16.7 Hours
Abstract
BACKGROUND
Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disorder caused by circulating autoantibodies against factor VIII (FVIII). In approximately 50% of the patients, the condition is associated with autoimmune diseases, cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid (BP) is a chronic autoimmune subepidermal blistering disease associated with tissue-bound and circulating autoantibodies against BP antigens 180 (BP180) and 230 (BP230). AHA-associated BP has a high mortality rate; hence, the understanding of this disease must improve.
CASE SUMMARY
A 69-year-old man presented with erythema, blisters, blood blisters, and crusts accompanied by severe pruritus for more than 20 days, and ecchymosis and swelling on his left upper arm for 3 days. Pathological examination revealed a subepidermal blister that contained eosinophils. Laboratory tests showed that the BP180 autoantibody levels had increased, isolated activated partial thromboplastin time was notably prolonged (115.6 s), and coagulation FVIII activity was extremely low (< 1.0%). Furthermore, the FVIII inhibitor titer had greatly increased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospital for effective treatment; however, he died after 2 days.
CONCLUSION
AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effective treatment are necessary.
Core Tip: Bullous pemphigoid associated with acquired hemophilia A is a rare disorder with unknown etiology and a high mortality rate. Because it develops in patients without a known bleeding disorder, clinicians should be aware of it and ensure its timely diagnosis and appropriate and effective treatment.
