Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Abstract

Pemphigus foliaceus (PF) is an autoimmune bullous dermatosis with anti-desmoglein-1 autoantibodies. Acquired hemophilia A (AHA) is a rare coagulation disorder with a high mortality rate, caused by anti-factor VIII immunoglobulin G antibodies leading to spontaneous severe hemorrhages into skin, muscles or soft tissues. This coagulopathy may be associated with malignancies, drug reactions and autoimmune disorders including bullous dermatoses. Herein, we demonstrate a first report of AHA in the course of pemphigus foliaceus. A 55-year-old woman presenting with extensive, erosive, crusted, scaly skin lesions was diagnosed with PF based on histopathological and immunofluorescent examination, confirmed by the presence of anti-desmoglein-1 antibodies on enzyme-linked immunoassay. She developed extensive internal hemorrhages and prolonged external bleeding after laparotomy. Based on coagulation tests, AHA was diagnosed. Simultaneous remission of pemphigus and coagulopathy occurred with immunosuppressants and recombinant activated factor VII.

Pemphigus foliaceus and acquired haemophilia: a rare but important association with life-threatening consequences

“…An association with autoimmune bullous dermatoses has been described in bullous pemphigoid, pemphigus vulgaris and linear IgA disease, but only one previous report of PF co‐existing with acquired haemophilia has been described . The features of reports associated with pemphigus subtypes are demonstrated in (Table ).…”

“…The features of reports associated with pemphigus subtypes are demonstrated in (Table ). This may occur due to crossreactions between certain skin antibodies and Factor VIII …”

Case Report of a Novel Association between Anti-p200 Pemphigoid and Acquired Haemophilia A

“…Although AHA is most commonly associated with autoimmune disease such as rheumatoid arthritis and systemic lupus erythematosus, malignancy and pregnancy, an association with AIBD of which bullous pemphigoid is most frequent, has been described 4 . Less commonly associated AIBDs include pemphigus 5 , other members of the pemphigoid group such as mucous membrane pemphigoid 6 7 , epidermolysis bullosa acquisita 8 , and linear IgA bullous dermatosis 9 . To our knowledge, this is the first reported case of anti-p200 pemphigoid co-existing with AHA.…”

“…One hypothesis is that of cross-reactivity of factor VIII inhibitor with various pathogenic domains of AIBD. Concurrent biochemical or clinical worsening of both AIBD and AHA have been reported in some cases 5 6 8 9 12 13 , while others do not demonstrate this relationship 7 11 . A recent retrospective evaluation of 6 patients with bullous pemphigoid and AHA did not demonstrate a temporal relationship between clinical and serological flare of bullous disease and activity of AHA 11 .…”