A case of epidermolysis bullosa acquisita with bleeding tendency due to factor VIII inhibitor (acquired haemophilia)

Abstract

10 Zomer SF, de Wit RF, van Bronswijk JE et al. Delusions of parasitosis. A psychiatric disorder to be treated by dermatologists? An analysis of 33 patients. Br J Dermatol 1998; 138: 1030-2.

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

“…The cases of coexisting AHA and acquired bullous epidermolysis can be caused by the potential cross-reaction between type VII collagen and fVIII. 11,14 By analogy, it may be speculated that the presented case of coexisting PF and AHA may be caused by the potential crossreaction between Dsg-1 and fVIII and increased synthesis of autoantibodies against fVIII with exacerbation of AHA might have been triggered by abrupt cessation of immunosuppressive therapy (methylprednisolone) by the patient.…”

“…Only a few cases of AHA coexisting with bullous pemphigoid, mucous membrane pemphigoid, pemphigus vulgaris, linear IgA bullous dermatosis (LABD) and epidermolysis bullosa acquisita have been reported. 2,5,[10][11][12][13][14][15] Coexistence of pemphigoid and AHA is believed to be caused by homologous structural sequences between plasma fVIII and hemidesmosomal antigen BPAG2. Antibodies against plasma fVIII are likely to interact with the central collagen-like domain of BPAG2; thus, a decrease in the level of fVIII inhibitor may improve cutaneous lesions of pemphigoid.…”

“…10,15 In LABD, autoantibodies react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1) which are fragments of the extracellular domain NC16A of BPAG2. 11 The NC16A region is thought to induce both the humoral and cellular autoimmune response and this is likely to contribute to the synthesis of fVIII inhibitor while the decreased activity of suppressor T lymphocytes additionally promotes the synthesis of autoantibodies. The cases of coexisting AHA and acquired bullous epidermolysis can be caused by the potential cross-reaction between type VII collagen and fVIII.…”

“…In fact, coexistence of PF and the congenital form of hemophilia A has been reported in a 3‐year‐old boy, but association with AHA has not been reported so far. Only a few cases of AHA coexisting with bullous pemphigoid, mucous membrane pemphigoid, pemphigus vulgaris, linear IgA bullous dermatosis (LABD) and epidermolysis bullosa acquisita have been reported . Coexistence of pemphigoid and AHA is believed to be caused by homologous structural sequences between plasma fVIII and hemidesmosomal antigen BPAG2.…”

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

“…2,19,[25][26][27][28] It is also reported that 2-8.3% of AH cases are associated with skin disorders: these include psoriasis vulgaris, bullous pemphigoid, exfoliative dermatitis, pemphigus vulgaris, erythema multiforme, erythema annulare centrifugum, scleroderma, malignant melanoma and nonspecific dermatoses. 2,6,7,19,25,26,29,30 Reported cases of autoimmune bullous dermatoses associated with AH include pemphigus vulgaris, [31][32][33] pemphigus erythematosus, 34 bullous pemphigoid, [35][36][37][38][39][40][41][42][43][44] epidermolysis bullosa acquisita [45][46][47] and anti-laminin 5 pemphigoid. 48 However, to the best of our knowledge, there have not been any previous reports of LABD associated with AH, so the present report may be the first case of LABD associated with AH.…”