Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy

Montagnon, Carmen M.; Lehman, Julia S.; Murrell, Dédée F.; Camilleri, Michael; Tolkachjov, Stanislav N.

doi:10.1016/j.jaad.2020.05.161

Cited by 17 publications

(26 citation statements)

References 130 publications

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“…Subepidermal autoimmune bullous diseases are a group of rare skin disorders characterized by autoantibodies against epidermal basement membrane zone (BMZ) proteins which include bullous pemphigoid, mucous membrane pemphigoid (MMP), anti-laminin (LM) γ1 pemphigoid and others ( 1 ).…”

Section: Introductionmentioning

confidence: 99%

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Case Report: Mucous Membrane Pemphigoid With IgG and IgA Anti-Laminin γ1 Antibodies and IgA Anti-Laminin α5 Antibodies

et al. 2022

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Mucous membrane pemphigoid (MMP) and anti-laminin (LM) γ1 pemphigoid, two subtypes of subepidermal autoimmune bullous diseases characterized by autoantibodies against epidermal basement membrane zone proteins, mainly show mucosal and skin lesions, respectively. The known autoantigens of MMP includes BP180, BP230, LM332, integrin α6β4 and type VII collagen, and anti-LMγ1 pemphigoid targets LMγ1. In this study, we present an unique MMP case with oral mucosal lesions, which showed positive IgA signals on basement membrane zone in indirect immunofluorescence using normal human skin and on dermal side in indirect immunofluorescence using salt-split skin, positive IgA autoantibodies against LMγ1 by immunoblotting of epidermal extracts, positive IgA autoantibodies against LMα5 by immunoblotting of LM521 recombinant protein (rLM521) and positive IgG autoantibodies against LMγ1 by immunoblotting of rLM111 and rLM521 at first visit (Day 0). After therapy, further serological analyses of serum samples collected at Day 30 and Day 50 indicated that IgA autoantibodies against LMγ1 were likely to be pathogenic. These results suggest that LMγ1 is another autoantigen of MMP, and our patient might be the first reported case of anti-LMγ1 MMP.

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Section: Introductionmentioning

confidence: 99%

“…MMP affects one or more mucous membranes and occasionally involves the skin ( 1 ). Known MMP-related autoantigens are BP180, BP230, LM332, integrin α6β4 and type VII collagen ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Mucous Membrane Pemphigoid With IgG and IgA Anti-Laminin γ1 Antibodies and IgA Anti-Laminin α5 Antibodies

et al. 2022

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“… 3 , 5 The presence of an eosinophil-rich infiltrate also favors a histologic diagnosis of BP, whereas LABD and BSLE typically exhibit more neutrophils. 4 Although BP is mainly characterized by linear IgG and C3 binding along the BMZ, IgA can be present in BP and an immunological overlap between BP and LABD has been reported. 6 – 8 …”

Section: Discussionmentioning

confidence: 99%

Annular bullous pemphigoid: A case report and review of literature

Arès

Lim

Jung

et al. 2022

SAGE Open Medical Case Reports

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Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented. We present a rare case of annular bullous pemphigoid in a 50-year-old male and offer a brief review of the literature. Only five other case reports, including three in adults, have described this unusual presentation, which can mimic other autoimmune blistering diseases, including linear IgA bullous dermatosis and pemphigus herpetiformis. Therefore, histopathology and immunologic studies were essential in properly diagnosing this patient. Our case supports that annular blistering lesions can be a clinical variant of bullous pemphigoid.

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“…However, BP usually requires use of systemic steroids in conjunction with steroid-sparing agents depending on severity of disease and patient comorbidities: doxycycline, niacinamide, mycophenolate mofetil, azathioprine, dupilumab, rituximab, intravenous immunoglobulin. 17 Mucous Membrane Pemphigoid Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is much less common than BP but more likely to affect the vulva and mucous membranes and difficult to diagnose and treat. 1,17,18 Early MMP is characterized by pain and irritation of the oral, ocular, and genital mucosa.…”

Section: Bullous Pemphigoidmentioning

confidence: 99%

“…17 Mucous Membrane Pemphigoid Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is much less common than BP but more likely to affect the vulva and mucous membranes and difficult to diagnose and treat. 1,17,18 Early MMP is characterized by pain and irritation of the oral, ocular, and genital mucosa. 17 Blisters are less frequently seen in MMP as they rapidly progress to painful erosions.…”

Section: Bullous Pemphigoidmentioning

confidence: 99%