Epidermolysis bullosa acquisita associated with psoriasis vulgaris

Abstract

We report a case of epidermolysis bullosa acquisita (EBA) associated with psoriasis vulgaris. A 71-year-old woman with psoriasis vulgaris developed subepidermal blisters on the extremities. Direct immunofluorescence demonstrated linear deposit of IgG at the basement membrane zone, which bound to the dermal side of normal human skin split with 1 mol/L NaCl. Immunoblot analysis using recombinant full-length type VII collagen detected a 290-kDa band, confirming the diagnosis of EBA. A literature search for previous reports found a few cases of EBA associated with psoriasis, and all cases, including our own, presented with widespread inflammatory vesicles and bullae, and responded to conventional therapy with corticosteroids and immunosuppressive agents. This study suggests that western blotting using recombinant full-length type VII collagen could be useful for diagnosis of EBA, and that EBA associated with psoriasis may have a tendency to be the inflammatory type.

Epidermolysis bullosa acquisita: What’s new?

“…[53][54][55] EBA associated with systemic diseases have been also often reported, including rheumatoid arthritis and diabetes mellitus, as well as cryoglobulinemia and psoriasis. [56][57][58][59][60] An association between EBA and inflammatory bowel disease (IBD) has been extensively documented; in particular, Crohn's disease has been described in approximately 30% of EBA patients. [61][62][63][64][65] However, to date, their relevance for the pathogenesis of both IBD and EBA is still unclear.…”

Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature

“…Ulcerative colitis (UC) is also associated with autoantibodies against type VII collagen and with EBA, although with a lower frequency than for CD . Occasionally, EBA is associated with other systemic diseases, including rheumatoid arthritis, diabetes mellitus and psoriasis …”

“…5,6 Occasionally, EBA is associated with other systemic diseases, including rheumatoid arthritis, diabetes mellitus 1 and psoriasis. 7 IBD occurs in clinically immunocompetent individuals, and its characteristic symptoms arise from an aggressive, cytokine-driven, noninfectious inflammation of the gut. In particular, T cells and antigenpresenting cells produce pro-inflammatory cytokines, including interleukin-6 and tumour necrosis factor-a, which cause mucosal inflammation and destruction.…”

Autoantibodies against type XVII collagen C-terminal domain in a patient with bullous pemphigoid associated with psoriasis vulgaris

“…The combination of psoriasis with autoimmune subepidermal bullous disease, for instance BP, 1,2 linear IgA bullous dermatosis 6,7 or EBA, 8 has been reported. Recently, especially in the Japanese population, several cases of the combination of psoriasis with anti-p200 pemphigoid have been reported.…”

“…He had never received psoralen + ultraviolet (UV) A (PUVA) or narrowband UVB therapy. 4,6,10 Dose of test substance 6,10 Batch volume 6 Purity of test substance 6,10 Test substance vehicle 6 Length of exposure 6 Time of occlusion 6 Release of test substance from chamber 6 Enhancement of penetration 6 Method of evaluation [4][5][6][7] Time of evaluation after exposure 2,4,6,7 Humidity 6,10 Water vapour gradient 6 Impairment of skin barrier 6 Anatomical site 6 Intrinsic level of skin sensitivity 6 Booster effect 8 Individual variation 8,10 Skin temperature 6 Ultraviolet exposure 6 Age 6 Sex 6 Race 6 Circadian rhythms 10 Concomitant medications 10 Skin biopsies were taken from two psoriatic plaques, one with and one without a blister. Histopathology of the first biopsy showed subepidermal blistering with an abundant infiltrate mainly of neutrophils and lymphocytes, and a small number of eosinophils.…”