Blistering Disease: Clinical Pearls from Neil Korman, MD

  1. Blisters can be caused by infections, inflammation (including several autoimmune conditions) as well as physical and mechanical injury.
  2. Diagnostic testing to make a diagnosis of an autoimmune blistering disease includes biopsies for histology and direct immunofluorescence as well as serum studies for indirect immunofluorescence and/or ELISA studies.
  3. The pemphigus family of diseases are uncommon but can be quite severe and typically require treatment with aggressive systemic immunosuppressive agents.
  4. These agents include oral corticosteroids often in combination with mycophenolate mofetil, azathioprine and rituximab.
  5. There are several subepidermal blistering diseases including Bullous Pemphigoid,

Mucous Membrane Pemphigoid, Epidermolysis Bullosa Acquisita, Dermatitis Herpetiformis, Linear IgA Disease and Pemphigoid Gestationis.

  1. Bullous pemphigoid is typically a disease of the elderly and is the most common of the subepidermal autoimmune blistering diseases.
  2. Indirect immunofluorescence studies utilizing salt split skin are extremely helpful in distinguishing between bullous pemphigoid and epidermolysis bullosa acquisita.
  3. Patients with mucous membrane pemphigoid more commonly have IgA antibody responses than do patients with bullous pemphigoid.
  4. Rituximuab is becoming a more commonly used and very successful therapy in the treatment of most of the autoimmune blistering diseases except for those with an IgA antibody response.