At a Glance - Bullous pemphigoid vs pemphigus vulgaris

Dr Jean Watkins, a sessional GP in Hampshire

BULLOUS PEMPHIGOID


Clinical features

  • Uncommon chronic autoimmune disease.
  • Most common in middle-aged and elderly.
  • IgG immunoglobulin and activated T-lymphocytes attack components of the basement membrane, causing separation of keratinocytes from the dermis.
  • May present with itchy eczematous or urticarial rash prior to blistering.
  • Crops of tense blisters, filled with clear or blood-stained fluid. Lesions heal without scarring.
  • Localised or widespread.
  • Mucosal ulceration in 10- 25 per cent of cases.
  • Untreated may persist for months or years with remissions and exacerbations. Usually settles after about five years.
  • Death more likely in the debilitated, as the result of secondary infection or effects of high-dosage steroids.

Management

  • Confirm diagnosis before starting treatment by skin biopsy from edge of blister for histology and direct immunofluorescence.
  • If positive, blood test for indirect immunofluorescence.
  • Hospital admission for severe or widespread cases. Avoid physical skin trauma.
  • Systemic steroids - high dosage to bring condition under control before reduction to maintenance dosage, often for months or years.
  • Bisphosphonate to reduce the risk of osteoporosis in those taking steroids long term.
  • Antibiotics for secondary infection.
  • Topical corticosteroids for localised disease or in conjunction with systemic steroids.
  • Immunosuppressive agents, biologicals such as rituximab and IV immunoglobulin have also been tried.

PEMPHIGUS VULGARIS

Clinical features

  • Rare autoimmune disease.
  • Most common between 50 and 60 years of age.
  • IgG binds to the protein desmoglein causing separation of keratinocytes from each other at bottom of the epidermis forming blisters.
  • Mortality 5-15 per cent from infection, side-effects of steroids and debility.
  • Usually first presents with ulceration of mucous membranes.
  • Flaccid blisters develop in the skin. These quickly rupture leaving painful erosions.

Management

  • Confirm diagnosis before starting treatment by skin biopsy for histology and direct immunofluorescence.
  • Blood test for indirect immunofluorescence.
  • Hospital admission usually required.
  • High dosage systemic corticosteroids.
  • Bisphosphonate to reduce the risk of osteoporosis in those taking steroids long term.
  • Antibiotics for secondary infection.
  • Immunosuppressive agents - azathioprine or mycophenolate - as steroid-sparing agents.
  • Biologicals such as rituximab are now being tried.
  • IV immunoglobulin.

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