Table 1.
Reported cases of acquired hemophilia A associated with bullous pemphigoid in the literature.
| Number [Ref.] |
Age | Sex | Onset BP | Evolution of BP under treatment | Max. inhib. titre (BU/mL) | Treatment of AHA | Evolution of AHA under treatment |
|---|---|---|---|---|---|---|---|
| 1 [4] |
74 | M | Concurrently with AHA | Good | 110 | CS, CsA, AZA, CPA, BA, IVIg, FVIII | Clinical and biological remission |
| 2 [5] |
68 | M | 6 months before AHA | Rapid response to topical CS | >2 | CS | Clinical and biological remission without recurrence over 12 months |
| 3 [6] |
47 | F | 3 months before AHA | Stable remission | 2.04 | CS, CPA, PP | Life-threatening complications followed by stable remission |
| 4 [7] |
88 | M | Few days before AHA | Improved with systemic and topical CS, doxycycline, nicotinamide | (+) | CS, BA | Died shortly after diagnosis |
| 5 [8] |
65 | M | 2-3 months before AHA | AHA occurred at BP relapse | 2 | CS | Good |
| 6 [8] |
67 | F | 6 months before AHA | Relapsed after self-discontinuation | 76 | CS, CS pulse, CPA, FFP, FVIII | Good |
| 7 [9] |
78 | M | 4 months before AHA | Resolved with CS | 839 | CS, CPA, BA | Relapse 3 months after withdrawing of CPA because of severe neutropenia Remission obtained with CS alone for 12 months |
| 8 [10] |
71 | F | ND | ND | (+) | CS | Died of pulmonary embolism |
| 9 [11] |
49 | F | 7 months before AHA | Resolved with CS, CPA | 148 | CS, CPA, FFP, PE | Good |
| 10 [12] |
71 | M | Concurrently with AHA | Resolved with CS | 219 | CS, IVIg, cryoprecipitate, BA | ND; patient transferred to another hospital. |
| 11 [13] |
83 | F | 3 years before AHA | Controlled with topical CS but relapsed | 17 | CS, BA | Died of severe hemorrhage |
| 12 [14] |
84 | F | 2 months before AHA | ND | 29 | CS, CPA, BA | Good, but died of sepsis. |
| 13 [15] |
81 | F | 4 weeks before AHA | Slight improvement with topical CS | 7 | / | Good, but died of ischemic heart disease |
| 14 [16] |
68 | F | Concurrently with AHA | Resolved with topical CS | 1.4 | BA | Good |
| 15 [17] |
38 | F | Before. | ND | 2.44 | CS, BA | ND. |
| 16 [18] |
64 | M | 4 weeks before AHA | Improved with systemic and topical CS, doxycycline, nicotinamide | (+) | CS, rituximab, BA | Remission; relapse after a few months, multiple transfusions, died of myocardial infarction |
| 17 [19] |
24 | M | 2 years before AHA | Improved with CS | 256 | CS, CS pulse, CPA, PP, rituximab, BA | Improved after 2 months |
| 18 [20] |
72 | M | 9 months before AHA | Resolved with MTX and topical CS | 200 | CS, rituximab, BA | Complete remission |
| 19 [21] |
60 | F | Concurrently with AHA | Resolved | (+) | CS, CPA, FFP, BA, IVIg | Complete remission |
| 20 [22] |
88 | M | 4 months before AHA | Not improved with CS | 7 | CS, rituximab, FFP | Remission of BP and AHA, but died of severe pneumonia |
| 21 [23] |
49 | F | 4 months before AHA | Minimal response to CS and IVIg | 17 | CS, CPA, BA, FVIII | Complete remission |
| 22 [24] |
80 | F | 12 months before AHA | Resolved with CS before AH | 20 | CS | Biological remission, even after CS discontinuation |
| 23 [25] |
73 | M | Concurrently with AHA | Good | (+) | CS, CPA, Rituximab, IVIg | Complete remission |
| 24 [26] |
61 | M | 1 month before AHA | Good | 32 | CS, BA | Clinical and biological improvement |
| 25 [∗] |
75 | M | 21 months before AHA | Controlled with systemic and topical CS + AZA/MMF | 25 | CS, Rituximab, BA | Complete remission |
The cases are presented in order of publication date. ND: not described; gender: M(ale)/F(emale); CS: corticosteroid; CsA: ciclosporin; AZA: azathioprine; CPA: cyclophosphamide; FFP: fresh frozen plasma; PE: plasma exchange; PP: plasmapheresis; BA: bypassing agents, for example, FEIBA (Factor Eight Inhibitor Bypassing Activity) or rFVII (recombinant Factor Seven); MTX: methotrexate; ∗: our case report.