Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

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.