Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Changing Clinical and Laboratory Concepts in Inherited Epidermolysis Bullosa

Changing Clinical and Laboratory Concepts in Inherited Epidermolysis Bullosa Abstract Within the past five years, our understanding of inherited epidermolysis bullosa (EB) has grown tremendously as a result of a combination of favorable conditions. First, recent advances in the fields of cell biology, applied immunology, and molecular biology have led to the development of in vitro models and in vivo markers for specific subtypes of EB. As a result, more refined and selective immunohistochemical reagents, notably polyclonal and monoclonal antibodies, are now available for diagnosis, both postnatal and prenatal, and for more basic studies aimed at elucidation of further mechanistic aspects of blister formation in this disease (J.D.F., R. Eady, MBBS, FRCP, M. Levy, PhD, unpublished observation, 1988).1-12 Eventually, such work should result in definition of the actual causative gene defects in EB. Second, recent laboratory findings have led to the development of new treatments (ie, phenytoin, autologous epidermal cell culture-derived skin grafts) and the initiation of additional References 1. Fine JD: Altered skin basement membrane antigenicity in epidermolysis bullosa . Curr Probl Dermatol 1987;17:111-126. 2. Eady RAJ, Tidman MJ, Heagerty AHM, et al: Approaches to the study of epidermolysis bullosa . Curr Probl Dermatol 1987;17:127-141. 3. Hintner H, Stingl G, Schuler G, et al: Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in mechanobullous diseases . J Invest Dermatol 1981; 76:119-124.Crossref 4. Fine JD, Gay S: LDA-1 monoclonal antibody: An excellent reagent for immunofluorescence mapping studies in patients with epidermolysis bullosa . Arch Dermatol 1986;122:48-51.Crossref 5. Fine JD, Breathnach S, Hintner H, et al: KF-1 monoclonal antibody defines a specific basement membrane antigenic defect in dystrophic forms of epidermolysis bullosa . J Invest Dermatol 1984;82:35-38.Crossref 6. Goldsmith L, Briggaman R: Monoclonal antibodies to anchoring fibrils for the diagnosis of epidermolysis bullosa . J Invest Dermatol 1983;81:464-466.Crossref 7. Heagerty AHM, Kennedy AR, Leigh IM, et al: Identification of an epidermal basement membrane defect in recessive forms of dystrophic epidermolysis bullosa by LH 7:2 monoclonal antibody: Use in diagnosis . Br J Dermatol 1986;115:125-131.Crossref 8. Kennedy AR, Heagerty AHM, Ortonne J-P, et al: Abnormal binding of an anti-amnion antibody to epidermal basement membrane provides a novel diagnostic probe for junctional epidermolysis bullosa . Br J Dermatol 1985;113:651-659.Crossref 9. Heagerty AHM, Kennedy AR, Eady RAJ, et al: GB3 monoclonal antibody for diagnosis of junctional epidermolysis bullosa . Lancet 1986;1:860.Crossref 10. Heagerty AHM, Kennedy AR, Gunner DB, et al: Rapid prenatal diagnosis and exclusion of epidermolysis bullosa using novel antibody probes . J Invest Dermatol 1986;86:603-605.Crossref 11. Heagerty AHM, Eady RAJ, Kennedy AR, et al: Rapid prenatal diagnosis of epidermolysis bullosa letalis using GB3 monoclonal antibody . Br J Dermatol 1987;117:271-275.Crossref 12. Fine JD, Couchman J: 19-DEJ-1 monoclonal antibody defines a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa . Clin Res 1988; 36:84A. 13. Bauer EA, Cooper TW, Tucker DR, et al: Phenytoin therapy of recessive dystrophic epidermolysis bullosa: Clinical trial and proposed mechanism of action on collagenase . N Engl J Med 1980;303:776-781.Crossref 14. Fine JD, Johnson L: Efficacy of systemic phenytoin in the treatment of junctional epidermolysis bullosa (JEB) . J Clin Pharmacol 1987;27:723. 15. Carter DM, Lin AN, Varghese MC, et al: Treatment of junctional epidermolysis bullosa with epidermal autografts . J Am Acad Dermatol 1987;17:246-250.Crossref 16. Fine JD, Johnson L: Efficacy of topical parfenac (bufexamac) in epidermolysis bullosa simplex: Results of a double-blind placebo-controlled crossover trial . J Clin Pharmacol 1987;27:723. 17. Carter DM, Caldwell D, Varghese M, et al: Effectiveness of bactroban ointment in chronic skin infection: Report of a clinical study , in Dobson RL, Leyden J, Noble WC (eds): Bactroban, Proceedings of an International Symposium . Princeton, NJ, Excerpta Medica, 1985, pp 228-240. 18. Haber RM, Hanna W, Ramsay CA, et al: Cicatricial junctional epidermolysis bullosa . J Am Acad Dermatol 1985;12:836-844.Crossref 19. Fine JD, Osment LS, Gay S: Dystrophic epidermolysis bullosa: A new variant characterized by progressive symmetrical centripetal involvement with scarring . Arch Dermatol 1985; 121:1014-1017.Crossref 20. Niemi K-M, Somer H, Kero M, et al: Epidermolysis bullosa simplex associated with muscular dystrophy with recessive inheritance . Arch Dermatol 1988;124:551-554.Crossref 21. Hashimoto K, Matsumoto M, Iacobelli D: Transient bullous dermolysis of the newborn . Arch Dermatol 1985;121:1429-1438.Crossref 22. Briggaman RA: Hereditary epidermolysis bullosa with special emphasis on newly recognized syndromes and complications . Dermatol Clin 1983;1:263-280. 23. Fine JD: Epidermolysis bullosa: Clinical aspects, pathology, and recent advances in research . Int J Dermatol 1986;25:143-157.Crossref 24. Bauer EA: Abnormalities in collagenase expression as in vitro markers for recessive dystrophic epidermolysis bullosa . J Invest Dermatol 1982;79( (suppl 1) ):105s-108s.Crossref 25. Tidman MJ, Eady RAJ: Evaluation of anchoring fibrils and other components of the dermal-epidermal junction in dystrophic epidermolysis bullosa by a quantitative ultrastructural technique . J Invest Dermatol 1985;84:374-377.Crossref 26. Briggaman RA: Is there any specificity to defects of anchoring fibrils in epidermolysis bullosa dystrophica, and what does this mean in terms of pathogenesis? J Invest Dermatol 1985; 84:371-373.Crossref 27. Fine JD, Griffith RD: A specific defect in glycosylation of epidermal cell membranes: Definition in skin from patients with epidermolysis bullosa simplex . Arch Dermatol 1985;121:1292-1296.Crossref 28. Fine JD: Epidermolysis bullosa: Variability of expression of cicatricial pemphigoid, bullous pemphigoid, and epidermolysis bullosa acquisita antigens . J Invest Dermatol 1985;85:47-49.Crossref 29. Sanchez G, Seltzer JL, Eisen AZ, et al: Generalized dominant epidermolysis bullosa simplex: Decreased activity of a gelatinolytic protease in cultured fibroblasts as a phenotypic marker . J Invest Dermatol 1983;81:576-579.Crossref 30. Winberg J-O, Gedde-Dahl T: Gelatinase expression in generalized epidermolysis bullosa simplex fibroblasts . J Invest Dermatol 1986;87:326-329.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Changing Clinical and Laboratory Concepts in Inherited Epidermolysis Bullosa

Fine, Jo-David
Archives of Dermatology , Volume 124 (4) – Apr 1, 1988
Download PDF
4 pages

Loading next page...
 
/lp/american-medical-association/changing-clinical-and-laboratory-concepts-in-inherited-epidermolysis-0qL6tdPkpr

References (34)

Publisher
American Medical Association
Copyright
Copyright © 1988 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1988.01670040025015
Publisher site
See Article on Publisher Site

Abstract

Abstract Within the past five years, our understanding of inherited epidermolysis bullosa (EB) has grown tremendously as a result of a combination of favorable conditions. First, recent advances in the fields of cell biology, applied immunology, and molecular biology have led to the development of in vitro models and in vivo markers for specific subtypes of EB. As a result, more refined and selective immunohistochemical reagents, notably polyclonal and monoclonal antibodies, are now available for diagnosis, both postnatal and prenatal, and for more basic studies aimed at elucidation of further mechanistic aspects of blister formation in this disease (J.D.F., R. Eady, MBBS, FRCP, M. Levy, PhD, unpublished observation, 1988).1-12 Eventually, such work should result in definition of the actual causative gene defects in EB. Second, recent laboratory findings have led to the development of new treatments (ie, phenytoin, autologous epidermal cell culture-derived skin grafts) and the initiation of additional References 1. Fine JD: Altered skin basement membrane antigenicity in epidermolysis bullosa . Curr Probl Dermatol 1987;17:111-126. 2. Eady RAJ, Tidman MJ, Heagerty AHM, et al: Approaches to the study of epidermolysis bullosa . Curr Probl Dermatol 1987;17:127-141. 3. Hintner H, Stingl G, Schuler G, et al: Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in mechanobullous diseases . J Invest Dermatol 1981; 76:119-124.Crossref 4. Fine JD, Gay S: LDA-1 monoclonal antibody: An excellent reagent for immunofluorescence mapping studies in patients with epidermolysis bullosa . Arch Dermatol 1986;122:48-51.Crossref 5. Fine JD, Breathnach S, Hintner H, et al: KF-1 monoclonal antibody defines a specific basement membrane antigenic defect in dystrophic forms of epidermolysis bullosa . J Invest Dermatol 1984;82:35-38.Crossref 6. Goldsmith L, Briggaman R: Monoclonal antibodies to anchoring fibrils for the diagnosis of epidermolysis bullosa . J Invest Dermatol 1983;81:464-466.Crossref 7. Heagerty AHM, Kennedy AR, Leigh IM, et al: Identification of an epidermal basement membrane defect in recessive forms of dystrophic epidermolysis bullosa by LH 7:2 monoclonal antibody: Use in diagnosis . Br J Dermatol 1986;115:125-131.Crossref 8. Kennedy AR, Heagerty AHM, Ortonne J-P, et al: Abnormal binding of an anti-amnion antibody to epidermal basement membrane provides a novel diagnostic probe for junctional epidermolysis bullosa . Br J Dermatol 1985;113:651-659.Crossref 9. Heagerty AHM, Kennedy AR, Eady RAJ, et al: GB3 monoclonal antibody for diagnosis of junctional epidermolysis bullosa . Lancet 1986;1:860.Crossref 10. Heagerty AHM, Kennedy AR, Gunner DB, et al: Rapid prenatal diagnosis and exclusion of epidermolysis bullosa using novel antibody probes . J Invest Dermatol 1986;86:603-605.Crossref 11. Heagerty AHM, Eady RAJ, Kennedy AR, et al: Rapid prenatal diagnosis of epidermolysis bullosa letalis using GB3 monoclonal antibody . Br J Dermatol 1987;117:271-275.Crossref 12. Fine JD, Couchman J: 19-DEJ-1 monoclonal antibody defines a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa . Clin Res 1988; 36:84A. 13. Bauer EA, Cooper TW, Tucker DR, et al: Phenytoin therapy of recessive dystrophic epidermolysis bullosa: Clinical trial and proposed mechanism of action on collagenase . N Engl J Med 1980;303:776-781.Crossref 14. Fine JD, Johnson L: Efficacy of systemic phenytoin in the treatment of junctional epidermolysis bullosa (JEB) . J Clin Pharmacol 1987;27:723. 15. Carter DM, Lin AN, Varghese MC, et al: Treatment of junctional epidermolysis bullosa with epidermal autografts . J Am Acad Dermatol 1987;17:246-250.Crossref 16. Fine JD, Johnson L: Efficacy of topical parfenac (bufexamac) in epidermolysis bullosa simplex: Results of a double-blind placebo-controlled crossover trial . J Clin Pharmacol 1987;27:723. 17. Carter DM, Caldwell D, Varghese M, et al: Effectiveness of bactroban ointment in chronic skin infection: Report of a clinical study , in Dobson RL, Leyden J, Noble WC (eds): Bactroban, Proceedings of an International Symposium . Princeton, NJ, Excerpta Medica, 1985, pp 228-240. 18. Haber RM, Hanna W, Ramsay CA, et al: Cicatricial junctional epidermolysis bullosa . J Am Acad Dermatol 1985;12:836-844.Crossref 19. Fine JD, Osment LS, Gay S: Dystrophic epidermolysis bullosa: A new variant characterized by progressive symmetrical centripetal involvement with scarring . Arch Dermatol 1985; 121:1014-1017.Crossref 20. Niemi K-M, Somer H, Kero M, et al: Epidermolysis bullosa simplex associated with muscular dystrophy with recessive inheritance . Arch Dermatol 1988;124:551-554.Crossref 21. Hashimoto K, Matsumoto M, Iacobelli D: Transient bullous dermolysis of the newborn . Arch Dermatol 1985;121:1429-1438.Crossref 22. Briggaman RA: Hereditary epidermolysis bullosa with special emphasis on newly recognized syndromes and complications . Dermatol Clin 1983;1:263-280. 23. Fine JD: Epidermolysis bullosa: Clinical aspects, pathology, and recent advances in research . Int J Dermatol 1986;25:143-157.Crossref 24. Bauer EA: Abnormalities in collagenase expression as in vitro markers for recessive dystrophic epidermolysis bullosa . J Invest Dermatol 1982;79( (suppl 1) ):105s-108s.Crossref 25. Tidman MJ, Eady RAJ: Evaluation of anchoring fibrils and other components of the dermal-epidermal junction in dystrophic epidermolysis bullosa by a quantitative ultrastructural technique . J Invest Dermatol 1985;84:374-377.Crossref 26. Briggaman RA: Is there any specificity to defects of anchoring fibrils in epidermolysis bullosa dystrophica, and what does this mean in terms of pathogenesis? J Invest Dermatol 1985; 84:371-373.Crossref 27. Fine JD, Griffith RD: A specific defect in glycosylation of epidermal cell membranes: Definition in skin from patients with epidermolysis bullosa simplex . Arch Dermatol 1985;121:1292-1296.Crossref 28. Fine JD: Epidermolysis bullosa: Variability of expression of cicatricial pemphigoid, bullous pemphigoid, and epidermolysis bullosa acquisita antigens . J Invest Dermatol 1985;85:47-49.Crossref 29. Sanchez G, Seltzer JL, Eisen AZ, et al: Generalized dominant epidermolysis bullosa simplex: Decreased activity of a gelatinolytic protease in cultured fibroblasts as a phenotypic marker . J Invest Dermatol 1983;81:576-579.Crossref 30. Winberg J-O, Gedde-Dahl T: Gelatinase expression in generalized epidermolysis bullosa simplex fibroblasts . J Invest Dermatol 1986;87:326-329.Crossref

Journal

Archives of Dermatology – American Medical Association

Published: Apr 1, 1988

There are no references for this article.