Association of bullous pemphigoid with acquired hemophilia A: a case report.
Pan Afr Med J
; 48: 27, 2024.
Article
em En
| MEDLINE
| ID: mdl-39220555
ABSTRACT
Acquired hemophilia A, a rare condition resulting in spontaneous bleeding without prior bleeding disorders, arises due to autoantibody-mediated inhibition of coagulation factor VIII and is typically associated with autoimmune, neoplastic, drug, or obstetric factors. We present the case of a 31-year-old woman with bullous pemphigoid, managed with corticosteroids since 2013, who presented spontaneous hemorrhagic manifestations. Upon admission, laboratory tests revealed hypochromic microcytic anemia, prolonged activated partial thromboplastin time, and a factor VIII level < 1%, indicative of acquired hemophilia A. Further assessments showed elevated Ristocetin cofactor activity, von Willebrand factor antigen, and a factor VIII inhibitor level of 665 BU. This underscores the importance of considering acquired hemophilia A in autoimmune dermatological conditions like bullous pemphigoid, highlighting the association between autoimmune disorders and coagulation abnormalities, particularly in cases of spontaneous hemorrhagic events.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Penfigoide Bolhoso
/
Hemofilia A
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Pan Afr Med J
/
Pan Afr. med. j
/
The Pan African medical journal
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Marrocos