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.
