Immunobullous conditions

LAST UPDATED: Apr 11, 2023

Introduction

The immunobullous disorders represent a group of conditions characterised by an antibody‐mediated autoimmune response against structural elements of the skin resulting in blistering of the skin and mucosae. Antibody targets include proteins in hemidesmosomes and the basement membrane zone (pemphigoid group), anchoring fibrils in the papillary dermis (epidermolysis bullosa acquisita), desmosomes (pemphigus group) and epidermal transglutaminase (dermatitis herpetiformis). If left untreated, immunobullous disorders may be associated with significant morbidity and mortality so prompt, accurate diagnosis and treatment are essential.

This chapter, which is set out as follows, provides only a brief overview, signposting to more detailed chapters:


History


Clinical findings

Presentation

Immunobullous conditions present in a variety of ways including:

  • Intense itch without obvious blisters (eg bullous pemphigoid in the pre-blistering phase) 
  • Tense blisters (eg bullous pemphigoid)
  • Vesicles i.e. small blisters (eg dermatitis herpetiformis - although in many patients the vesicles are hard to see due to scratching)
  • Erosions and crusting (eg pemphigus vulgaris)
  • Oral and/or other mucosal involvement (eg mucous membrane pemphigoid)
Detailed information on immunobullous conditions
  • Refer to the related chapters at the top of this page
Additional resources

The number of immunobullous conditions is extensive, for more information on diagnostics, other conditions, and further images please refer to the following resources:


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