This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (14)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Definition in Skin From Patients With Epidermolysis Bullosa Simplex

Jo-David Fine, MD; Robert D. Griffith, MD

Arch Dermatol. 1985;121(10):1292-1296.

Abstract
• Why blister formation occurs within the epidermis in epidermolysis bullosa (EB) simplex is not known. One possibility is that there are diminished amounts, absence, or biochemical alterations of one or more structural components of epidermal cell membranes, thereby leading to increased skin fragility. In order to test this hypothesis, biopsy specimens were obtained from clinically normal-appearing skin of patients with simplex, junctional, and dystrophic forms of EB and normal adult volunteers. Immunofluorescence studies were performed on each specimen using eight fluorescein-labeled affinity-purified lectins shown to bind uniformly to epidermal cell membranes of normal human adult skin and neonatal foreskin. To examine the epidermal cytoskeleton, each tissue specimen was also examined using two antikeratin monoclonal antibodies. Irregular and focally granular epidermal membrane staining was noted in each EB simplex specimen examined with the lectin-peanut agglutinin. In contrast, uniformly crisp membrane staining was seen in each specimen from patients with junctional or dystrophic EB and from normal volunteers. This epidermal cell membrane glycosylation defect appears to have the restricted carbohydrate specificity of peanut agglutinin since staining of EB simplex skin with each of the remaining seven lectins was indistinguishable from that seen in skin from patients with the other forms of EB and normal adult skin. Furthermore, the epidermis in EB simplex skin appears to be selectively abnormal since the same tissue specimens demonstrated normal keratin cytoskeleton staining.

(Arch Dermatol 1985;121:1292-1296)

Author Affiliations
From the Department of Dermatology, University of Alabama at Birmingham School of Medicine (Drs Fine and Griffith) and the Dermatology Service, Birmingham Veterans Administration Medical Center (Dr Fine).

Footnotes
Accepted for publication May 31, 1985.

Reprint requests to Department of Dermatology, University of Alabama at Birmingham, University Station, Box 76, Birmingham, AL 35294 (Dr Fine).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Recessive Dystrophic Epidermolysis Bullosa Skin Displays a Chronic Growth-Activated Immunophenotype: Implications for Carcinogenesis
Smoller et al.
Arch Dermatol 1990;126:78-83.
ABSTRACT  

Epidermolysis Bullosa Simplex Responding to Isotretinoin
Andreano and Tomecki
Arch Dermatol 1988;124:1445-1446.
ABSTRACT  

Changing Clinical and Laboratory Concepts in Inherited Epidermolysis Bullosa
Fine
Arch Dermatol 1988;124:523-526.
ABSTRACT