Ann Dermatol.  1996 Apr;8(2):85-89. 10.5021/ad.1996.8.2.85.

Clinical Features of Epidermolysis Bullosa Acquisita

• Lee CW

• KMID: 2360536
• DOI: http://doi.org/10.5021/ad.1996.8.2.85

Abstract

Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease of the skin occurring mostly in middle-aged persons with characteristic skin lesions of inflammatory in a vesiculobullae and mechanobullous lesions. Separation of the skin occurs at the dermoepidermal junction (DEJ) initiated by an immune process involving the anchoring fibrils (AF) 764, which have a role in the normal adherence of the epidermis and the dermis. Patients with EBA have autoantibodies of IgG to type VII collagen which is the main component of AF. An electron microscopic. picture of normal DEJ is shown in figure 1, and the antigen site 2011s of this disease (AF) is noted at the upper-most part of the dermis. In EBA, a biopsy specimen shows subepidermal bulla with a variable degree of dermal in-filtrates. Immunofluorescence (IF) demonstrates a linear deposit of IgG. The pattern of immune deposits along the DEJ is similar to that of bullous pemphigoid. However, the linear iing. fashion is thicker and coarser. When examined by the indirect method with a semi-horizontal section of normal human skin substrates the same patterns can be observed: a fine linear deposit with bullous pemphigoid antibodies and a slightly coarser linear pattern with EBA antibodies. With salt-split skin substrates, the serum autoantibodies of IgG are found to be bound only to the dermal side, the AF zone (Fig. 2). This immunopathologic study can provide a diagnostic finding. Transmission electron microscopic examination reveals blister to be localized just beneath the lamina densa, the site of the immune deposit. In immunoblot analysis of the patient's serum against the. dermal extracts, serum antibodies are found to recognize type VII collagen of 290/145 kD (Fig. 3). This is a confirmatory technique (with antibody-positive sera) in the diagnosis of EBA.

MeSH Terms

Antibodies
Autoantibodies
Biopsy
Blister
Collagen Type VII
Dermis
Diagnosis
Epidermis
Epidermolysis Bullosa Acquisita*
Epidermolysis Bullosa*
Fluorescent Antibody Technique
Humans
Immunoglobulin G
Methods
Pemphigoid, Bullous
Skin
Antibodies
Autoantibodies
Collagen Type VII
Immunoglobulin G