Rare Autoimmune Blistering Disorders

  • Christine S. Ahn
  • William W. HuangEmail author


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.


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



Bullous pemphigoid


Basement membrane zone


Bullous pemphigoid 180 antigen


Bullous pemphigoid 230 antigen


Complement component 3


Direct immunofluorescence


Dermoepidermal junction








Epidermolysis bullosa acquisita


Enzyme-linked immunosorbent assay


Hematoxylin and eosin


Immunoglobulin A


Intraepidermal neutrophilic


Immunoglobulin G


Indirect immunofluorescence


Lichen planus


Lichen planus pemphigoides




Psoralen plus ultraviolet A


Systemic lupus erythematosus


Subcorneal pustular dermatosis


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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