Bullous Skin Diseases

Skin and Mucous Membrane Blisters in Autoimmune Disease

The autoimmune bullous skin disorders may occur alone, for instance in patients with pemphigus, or in people with other autoimmune disorders, such as systemic lupus erythematosus (SLE). Bullous skin disorders may also occasionally occur in pregnancy.

Symptoms

Bullous skin diseases are characterized by the presence of blisters or erosions of the skin and mucous membranes. In some bullous disorders such as pemphigus vulgaris, blistering is the primary disease manifestation. In other disorders, such as lichen planus or systemic lupus erythematosus (SLE), blisters occur infrequently. Bullous SLE occurs in less than 5 percent of patients, and some studies suggest that there is an increased incidence of the bullous variant of SLE in young black women.

Acquired, Induced, or Autoimmune

Bullous skin diseases may be acquired or induced or they may be autoimmune in origin. The autoimmune bullous skin disorders are all characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis and dermoepidermal basement membrane zone. The consequences of these antibodies are a loss of the targeted protein's adhesive properties, which leads, in turn, to the appearance of blisters and erosions. Like most autoimmune disorders, autoimmune bullous or vesiculobullous skin disorders are more likely to occur in women. Women of childbearing age have the highest risk for developing autoimmune bullous diseases.

The autoimmune bullous disorders include: 

  Pemphigus & Pemphigoid disorders
  Epidermolysis bullosa acquisita 
  Dermatitis herpetiformis 
  Herpes (pemphigoid) gestationis 
  IGA -mediated disorders

Pemphigus and Pemphigoid Disorders

Pemphigus disorders and pemphigoid disorders (bullous pemphigoid, cicatrical pemphigoid) are considered prototypic bullous disorders because of their well-defined autoantibody-mediated development or pathogenesis. The three pemphigus disorders target desmoglein protein, and the pemphigoid disorders target Type XVII collagen and bullous pemphigoid (BP) antigen 2, BP antigen 180, BP antigen 230, and laminin 5. The end result is a loss of the skin's architecture. In the absence of a well-organized viable skin composition, blisters and erosions form craters between the tissues cells.

Pemphigus disorders may present or worsen during pregnancy, especially in the first and second trimesters. Menstruation is reported to trigger or cause relapses of pemphigus. Other suspected risk factors for pemphigus include pregnancy, frequent handling of certain spices, repeated minor thermal burns, and cosmetic procedures.

Bullous pemphigoid primarily affects the skin, especially the lower abdomen, groin, and flexor surfaces of the hands and feet. About 10-40 percent of patients have mucous membrane involvement. The common age of onset is 65 to 75 years.

Cicatricial pemphigoid (CP) usually targets people between the ages of 60-80 although it has been reported in people of all ages, including children. Several subgroups of CP have been described including antiepiligrin CP, Pure Ocular CP, which affects only the eyes, Anti-BP Antigen Mucosal Pemphigoid, and Oral Pemphigoid.

Dermatitis Herpetiformis and IgA-mediated Autoimmune Disorders

Dermatitis herpetiformis is a bullous skin disorder that occurs in patients with gluten sensitivity. It may also occur in patients with autoimmune thyroid disease, type 1 diabetes, vitiligo, and rheumatoid arthritis. The blisters usually appear on the buttocks and back and are known to cause severe itching.