Pemphigoid (bullous)

This acquired autoimmune blistering disorder is characterized by large, tense, subepidermal blisters on an erythematous base.

Autoantibodies (mainly IgG) targeting the components of the basement membrane can be detected (1).

Usually it is a disease of the elderly (2), rarely presenting before 50 years of age. In the UK, it is more common than pemphigus with an annual incidence of 1 per 10000.

Both men and women are affected equally (1). Pemphigoid may be associated with malignancy although this is disputed in recent studies (1).

It is the most common cutaneous subepidermal bullous autoimmune disorder (2). There is a high mortality rate associated with this disease (3).

Pemphigoid may be associated with malignancy although this is disputed in recent studies.

Click here for an example image of bullous pemphigoid

Drug-associated bullous pemphigoid (4):

• drugs associated with development of bullous pemphigoid include:
  • antibiotics
  • beta-blockers
  • non-steroidal anti-inflammatory drugs (NSAIDs)
  • diuretics
  • anti-tumor necrosis factor (TNF)-alpha
  • dipeptidyl peptidase 4 inhibitors (DPP-4i)
  • immune checkpoint inhibitors targeting programmed cell death receptor 1 (PD-1) and its ligand (PD-L1)

High potency topical steroids and systemic steroids are the current mainstay of therapy (5).

Reference:

• 1. British Association of Dermatologists 2002. Guidelines for the management of bullous pemphigoid
• 2. Beissert S et al. A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid. Arch Dermatol. 2007; 143(12):1536-1542
• 3. Langan S.M. et al. Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study. BMJ 2008; 337:a180
• 4.Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G. Bullous Pemphigoid: Trigger and Predisposing Factors. Biomolecules. 2020 Oct 10;10(10):1432. doi: 10.3390/biom10101432
• 5. Miyamoto D, Santi CG, Aoki V, Maruta CW. Bullous pemphigoid. An Bras Dermatol. 2019 Mar-Apr;94(2):133-146. doi: 10.1590/abd1806-4841.20199007