Detection of linear IgE deposits in bullous pemphigoid and mucous membrane pemphigoid: a useful clue for diagnosis

Abstract

Our findings indicate that demonstration of tissue-bound IgE deposits provides an additional useful criterion for diagnosis of BP and MMP in some patients. Prospective studies are needed to better correlate the presence of tissue-bound and circulating IgE autoantibodies and their specificity with distinct clinical features and course of BP and MMP.

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

“…[82,83] Provost and Tomasi first described weak linear IgE deposition along the BMZ in 25% of patients, [82] …”

“…[79] The percentage of BP patients with IgE anti-BP180 NC16A autoantibodies varied substantially in the different reports. [46,83,95,96,[99][100][101][102][103][104][105][106] In the initial report, we identified anti-BP180 NC16A IgE reactivity in 55% of 18 BP sera. [96] Subsequent studies found IgE anti-NC16A in 100% of 10, 83% of 18, 22% of 37, 77% of 43, 30% of 67, 61% of 31, and 71% of 56 BP patients.…”

“…[88] In contrast, in 7.7% of 299 pregnant women, anti-BP180 IgE was detected and was shown to label both the cutaneous BMZ and the placental amnion by indirect IF microscopy as well as cultured cytothrophoblasts. [126] IgE binding along the BMZ in the patients' skin was found in 69% of 13 mucous membrane pemphigoid patients [83] but in none of 2 and 12 patients with pemphigoid gestationis. [127,128] Pemphigus Elevated total IgE serum levels were found in patients with endemic pemphigus foliaceus but rarely in pemphigus vulgaris.…”

Functional Characterization of an IgE-Class Monoclonal Antibody Specific for the Bullous Pemphigoid Autoantigen, BP180

“…In BP, both IgG-and IgE-class autoantibodies specific for BP180 are found in the circulation and bound to the basement membrane zone (BMZ) of affected skin. (1)(2)(3)(4) These two classes of autoantibodies, which have been shown to target the same cluster of epitopes located within the non-collagenous 16A region (NC16A) of BP180, are produced by the vast majority of BP patients (IgG, 94% of patients; IgE, > 85%). (1,(5)(6)(7) Early work probing the pathogenesis of BP in in vitro and in vivo models demonstrated a role for IgG class autoantibodies (6,(8)(9)(10) ; however, none of these IgG-based models were able to recapitulate the early phase of lesion development in the human disease.…”

Is anti-BP180 IgE associated with clinical phenotype? Reply to the letter to the editor

“…Although the association with tissue bound IgE with urticarial papules and plaques in patients with bullous pemphigoid have been reported [4], Moriuchi et al reported there were no differences between in vivo IgE-positive and IgE-negative cases, not only in disease severity but also in clinical course and outcome [5]. However, these studies were performed by direct immunofluorescence and the IgE antibodies were tissue-bound, not circulating autoantibodies.…”