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Rare Autoimmune Blistering Disorders

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Autoimmune Bullous Diseases

Abstract

The spectrum of autoimmune blistering disorders continues to evolve as previous associations become new entities, and these entities demonstrate distinct clinical, histologic, and immunohistochemical characteristics. The rare autoimmune blistering disorders present both diagnostic and therapeutic challenges to clinicians. Diagnostically, there can be overlapping features between the rare and more common autoimmune diseases. From a therapeutic standpoint, there is a general lack of studies that demonstrate treatment efficacy and outcomes in these entities leading to clinical practice gaps. This chapter will review the clinical and histological features of lichen planus pemphigoides (LPP), bullous lichen planus (BLP), bullous systemic lupus erythematosus (SLE), IgA pemphigus, and subcorneal pustular dermatosis (SPD), and provide an evidence-based review of the treatment options reported in the literature.

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Abbreviations

BP:

Bullous pemphigoid

BMZ:

Basement membrane zone

BP180:

Bullous pemphigoid 180 antigen

BP230:

Bullous pemphigoid 230 antigen

C3:

Complement component 3

DIF:

Direct immunofluorescence

DEJ:

Dermoepidermal junction

Dsc1:

Desmocollin-1

Dsg1:

Desmoglein-1

Dsg3:

Desmoglein-3

EBA:

Epidermolysis bullosa acquisita

ELISA:

Enzyme-linked immunosorbent assay

H&E:

Hematoxylin and eosin

IgA:

Immunoglobulin A

IEN:

Intraepidermal neutrophilic

IgG:

Immunoglobulin G

IIF:

Indirect immunofluorescence

LP:

Lichen planus

LPP:

Lichen planus pemphigoides

NC:

Non-collagenous

PUVA:

Psoralen plus ultraviolet A

SLE:

Systemic lupus erythematosus

SPD:

Subcorneal pustular dermatosis

TNF:

Tumor necrosis factor

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Authors and Affiliations

  1. Department of Dermatology, Wake Forest University School of Medicine, 4618 Country Club Road, Winston Salem, NC, 27104, USA

    Christine S. Ahn MD & William W. Huang MD, MPH

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  1. Christine S. Ahn MD
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  2. William W. Huang MD, MPH
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Correspondence to William W. Huang MD, MPH .

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Editors and Affiliations

  1. Department of Dermatology, University of Alabama at Birmingham, BIRMINGHAM, Alabama, USA

    Naveed Sami

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Ahn, C.S., Huang, W.W. (2016). Rare Autoimmune Blistering Disorders. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_11

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  • DOI: https://doi.org/10.1007/978-3-319-26728-9_11

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