Acquired Haemophilia Associated with Urticarial Vasculitis

Area of Science:

• Autoimmune diseases
• Hematology
• Immunology

Background:

• Acquired haemophilia (AHA) is a rare autoimmune disorder characterized by autoantibodies inhibiting factor VIII (FVIII) activity.
• The incidence of acquired FVIII inhibitors is approximately 1 case per million per year.
• AHA is associated with various conditions including autoimmune diseases, malignancies, drug treatments, pregnancy, and infections.

Purpose of the Study:

• To report a rare case of acquired haemophilia (AHA) associated with urticarial vasculitis.
• To describe the clinical presentation and successful management of this rare condition.

Main Methods:

• Case report of a 59-year-old woman.
• Diagnosis of acquired haemophilia (AHA) and urticarial vasculitis.
• Treatment with factor eight inhibitor bypassing activity (FEIBA) and prednisolone.

Main Results:

• The patient presented with cutaneous and muscle haematomas.
• The condition was secondary to acquired haemophilia (AHA) in association with urticarial vasculitis.
• Successful treatment was achieved with FEIBA and prednisolone.

Conclusions:

• Acquired haemophilia (AHA) is a rare autoimmune disease.
• AHA can lead to severe, life-threatening hemorrhage.
• The association between AHA and urticarial vasculitis is exceptionally rare.