Acquired hemophilia associated with bullous pemphigoid: a case report

Int J Clin Exp Pathol. 2012;5(1):102-4. Epub 2012 Jan 5.

Abstract

The development of factor VIII inhibitors in non-hemophilic patients is rare and may occur in healthy individuals, mostly elderly and women in postpartum period, and in patients with malignant neoplasia or autoimmune diseases, such as bullous pemphigoid. We described the case of a 60-year-old female patient who developed bullous pemphigoid for 3 month and presented with bleeding tendency and hematoma in the tongue. Therapy with methylprednisolone, cyclophosphamide, intravenous immunoglobulin and factor VIII reposition was instituted, resulting in a remission of the bleeding and negativity for antibodies against factor VIII titers. We concluded that, despite its rarity, the presence of acquired factor VIII inhibitors should be investigated when patients with autoimmune diseases develop bleeding manifestations.

Keywords: Bullous pemphigoid; acquired hemophilia; factor VIII inhibitor.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Coagulants / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Erythrocyte Transfusion
  • Factor VIII / therapeutic use
  • Female
  • Fluorescent Antibody Technique
  • Hemophilia A / drug therapy
  • Hemophilia A / etiology*
  • Hemophilia A / physiopathology
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Pemphigoid, Bullous / complications*
  • Pemphigoid, Bullous / drug therapy
  • Pemphigoid, Bullous / physiopathology
  • Plasma

Substances

  • Anti-Inflammatory Agents
  • Coagulants
  • Immunoglobulins, Intravenous
  • Cyclophosphamide
  • Factor VIII
  • Methylprednisolone

Supplementary concepts

  • Factor 8 deficiency, acquired