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Rare Autoimmune Blistering Disorders

  • Christine S. Ahn
  • William W. HuangEmail author
Chapter

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.

Keywords

Autoimmune bullous Lichen planus pemphigoides Lichen planus Bullous pemphigoid IgA pemphigus Bullous lupus Subcorneal pustular dermosis Sneddon-Wilkinson 

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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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyWake Forest University School of MedicineWinston SalemUSA

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