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Epidermolysis bullosa acquisita

Evidence: Antibody

Names

Acquired Epidermolysis bullosa
Autoimmune Epidermolysis bullosa
EBA
Epidermolysis bullosa acquisita

Description

In epidermolysis bullosa acquisita (EBA) the skin and mucus membranes are chronically inflamed and may form blisters in response to minor injury. Common areas of blistering include the hands, feet, knees, elbows, and buttocks. It can also affect the mouth, nose, and eyes.  Evidence suggests an association between EBA and inflammatory bowel diseases, such as ulcerative colitis and Crohn’s disease. (IBD is reported to be present in approximately 30% of EBA patients (23, 34, 35).

There are two main types of EBA, non-inflammatory and non-inflammatory. The non-inflammatory type is more common and resembles the hereditary disease, epidermolysis bullosa. The inflammatory types resemble autoimmune bullous pemphigoid. Mucous Membrane EBA (MM-EBA) is a type of inflammatory EBA that affects mucous membranes.

EBA is marked by the presence of autoantibodies (mainly IgG class) to type VII collagen.

Patient Groups

None

Bloggers

None

Prevalence

US Cases: 6,400 [Zhu 1990]. 2 cases per 100,000.

Typical Age of onset

EBA can develop at any age, but onset peaks among patients in their 20s and 70s.

Symptoms

Symptoms vary from one patient to the next.

Non-inflammatory EBA:

  • tense, blood- or pus-filled blisters, mostly on the hands, knees, knuckles, elbows and ankles

  • mucous-membrane blisters that rupture easily

  • healing with significant scarring and small white spots (milia)

Inflammatory EBA:

  • widespread blisters that are not localized to trauma-prone sites

  • generalized redness and itching

  • healing with minimal scarring

Mucous membrane EBA:

  • blisters on various mucous membranes

  • significant scarring and dysfunction

EBA may mimic other inflammatory blistering diseases, most often bullous pemphigoid and bullous systemic lupus erythematosus (which is also associated with anti-Col7 autoantibodies).

In comparison to bullous pemphigoid, EBA:

  • Arises in younger individuals, usually under the age of 70 years

  • Has prominent involvement of the head and neck

  • Involves mucous membranes

Lesions heal with scarring and the formation of milia.