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Journal of the American Academy of Dermatology
Volume 50, n° 4
pages 572-581 (avril 2004)
Doi : 10.1016/j.jaad.2003.09.026
accepted : 13 September 2003
Relative extent of skin involvement in inherited epidermolysis bullosa (EB): composite regional anatomic diagrams based on the findings of the National EB Registry, 1986 to 2002
 

David T Devries, MD a, b, Lorraine B Johnson, MPH, ScD a, b, c, Madeline Weiner, RN a, b, Jo-David Fine, MD, MPH a, d, e, f,
a National Epidermolysis Bullosa Registry, Lexington, Kentucky, USA 
b Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA 
c Department of Public Health Nursing, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA 
d Department of Epidemiology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA 
e Department of Medicine (Dermatology), University of Kentucky College of Medicine, Lexington, Kentucky, USA 
f Dermatology Associates of Kentucky, Lexington, Kentucky, USA 

*Reprint requests: Jo-David Fine, MD, MPH, Dermatology Associates of Kentucky, PSC, 250 Fountain Ct, Lexington, KY 40509, USA.
Abstract
Background

Inherited epidermolysis bullosa (EB) encompasses 4 major types and at least 23 clinically distinctive phenotypes. Although considerable variability in cutaneous disease activity is known to exist within each, severity and anatomic distribution of skin lesions remain the major criteria used for subclassification.

Objective

We sought to generate accurate anatomic “density” diagrams depicting the relative extent and location of skin lesions within each major EB subtype.

Methods

Diagrams were created for each major EB type, on the basis of medical history and physical examination findings obtained from 1986 to 2002 from 3280 consecutive enrollees in the National EB Registry.

Results

An anatomic diagram was created for each of the major EB subtypes, representing a prototypic composite photograph of cutaneous disease activity.

Conclusions

Marked variability exists in the extent of skin involvement within each major EB subtype. The use of these diagrams, generated from the world's largest cohort of patients with EB, should assist the clinician in more accurately subclassifying newly encountered patients.

The full text of this article is available in PDF format.

Abbreviations : DDEB, EB, EBS, EBS-DM, EBS-K, EBS-WC, JEB, JEB-H, NEBR, NIH, RDEB, RDEB-I, RDEB-O



 Supported by the National Institutes of Health; Doris Duke Foundation Predoctoral Fellowship (Dr Devries); and National Institute of Arthritis, Musculoskeletal and Skin Diseases grants (NO1 AM62271, NO1 AR22200, NO1 AR22201, and NO1 AR72233) and Midcareer Investigator Award for Patient-oriented Research (K24 AR02098) (Dr Fine).
Conflicts of interest: None identified.
NEBR Collaborating Institutions and Investigators: Data coordinating centers: Rockefeller University, 1986-1992 (D. M. Carter); University of North Carolina at Chapel Hill, 1992-2002 (J-D. Fine). Clinical centers or subcontract sites for regional data collection: Rockefeller University (1986-1997; D. M. Carter); University of North Carolina at Chapel Hill (1990-2002; J-D. Fine); Stanford University (1989-2002; E. A. Bauer, J. McGuire); Washington University (1986-1989; E. A. Bauer); University of Alabama at Birmingham (1986-1990; J-D. Fine); University of Washington (1986-1997; V. P. Sybert); Children's Memorial Hospital, Chicago (1992-1995; A. Paller); University of Colorado (1992-1997; W. Weston).



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