Epidermolysis Bullosa Medication

  • Author: M Peter Marinkovich, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 22, 2010
 
 

Medication Summary

Epidermolysis bullosa (EB) is a genetic disease and no drugs are known to correct the underlying molecular defects. Prolonged use of steroids is contraindicated in the treatment of inherited forms of epidermolysis bullosa. Steroid-induced complications further warrant prohibiting their use. No other drugs, including phenytoin and tetracycline, have improved the blistering or epithelial disadhesion in epidermolysis bullosa significantly or consistently.

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Contributor Information and Disclosures
Author

M Peter Marinkovich, MD  Associate Professor, Department of Dermatology and Program in Epithelial Biology, Stanford University Medical Center; Dermatology Service, VA Palo Alto Health Care System

M Peter Marinkovich, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Jean Paul Ortonne, MD  Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France

Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Fine JD. Inherited epidermolysis bullosa: past, present, and future. Ann N Y Acad Sci. Apr 2010;1194:213-22. [Medline].

  2. Sawamura D, Nakano H, Matsuzaki Y. Overview of epidermolysis bullosa. J Dermatol. Mar 2010;37(3):214-9. [Medline].

  3. Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. Jun 2008;58(6):931-50. [Medline].

  4. McGrath JA, Mellerio JE. Epidermolysis bullosa. Br J Hosp Med (Lond). Apr 2006;67(4):188-91. [Medline].

  5. Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances and the Findings of the National Epidermolysis Bullosa Registry. Baltimore, Md: Johns Hopkins University Press; 1999.

  6. Risser J, Lewis K, Weinstock MA. Mortality of bullous skin disorders from 1979 through 2002 in the United States. Arch Dermatol. Sep 2009;145(9):1005-8. [Medline].

  7. Ersoy-Evans S, Erkin G, Fassihi H, et al. Ectodermal dysplasia-skin fragility syndrome resulting from a new homozygous mutation, 888delC, in the desmosomal protein plakophilin 1. J Am Acad Dermatol. Jul 2006;55(1):157-61. [Medline].

  8. Fassihi H, Eady RA, Mellerio JE, et al. Prenatal diagnosis for severe inherited skin disorders: 25 years' experience. Br J Dermatol. Jan 2006;154(1):106-13. [Medline].

  9. Fassihi H, Eady RA, Mellerio JE, et al. Prenatal diagnosis for severe inherited skin disorders: 25 years' experience. Br J Dermatol. Jan 2006;154(1):106-13. [Medline].

  10. Heagerty AH, Eady RA, Kennedy AR, et al. Rapid prenatal diagnosis of epidermolysis bullosa letalis using GB3 monoclonal antibody. Br J Dermatol. Sep 1987;117(3):271-5. [Medline].

  11. Marinkovich MP, Meneguzzi G, Burgeson RE, et al. Prenatal diagnosis of Herlitz junctional epidermolysis bullosa by amniocentesis. Prenat Diagn. Nov 1995;15(11):1027-34. [Medline].

  12. Ortiz-Urda S, Garcia J, Green CL, et al. Type VII collagen is required for Ras-driven human epidermal tumorigenesis. Science. Mar 18 2005;307(5716):1773-6. [Medline].

  13. Tong L, Hodgkins PR, Denyer J, et al. The eye in epidermolysis bullosa. Br J Ophthalmol. Mar 1999;83(3):323-6. [Medline].

  14. [Guideline] American Academy of Pediatric Dentistry Council on Clinical Affairs. Guideline on management of dental patients with special health care needs. Pediatr Dent. 2008-2009;30(7 Suppl):107-11. [Medline].

  15. Woodley DT, Keene DR, Atha T, Huang Y, Lipman K, Li W, et al. Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa. Nat Med. Jul 2004;10(7):693-5. [Medline].

  16. Woodley DT, Keene DR, Atha T, et al. Intradermal injection of lentiviral vectors corrects regenerated human dystrophic epidermolysis bullosa skin tissue in vivo. Mol Ther. Aug 2004;10(2):318-26. [Medline].

  17. Mavilio F, Pellegrini G, Ferrari S, et al. Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cells. Nat Med. Dec 2006;12(12):1397-402. [Medline].

  18. Bauer EA, Herron GS, Marinkovich MP, Khavari PA, Lane AT. Gene therapy for a lethal genetic blistering disease: a status report. Trans Am Clin Climatol Assoc. 1999;110:86-92. [Medline].

  19. Pulkkinen L, Uitto J. Mutation analysis and molecular genetics of epidermolysis bullosa. Matrix Biol. Feb 1999;18(1):29-42. [Medline].

  20. Vailly J, Gagnoux-Palacios L, Dell'Ambra E, et al. Corrective gene transfer of keratinocytes from patients with junctional epidermolysis bullosa restores assembly of hemidesmosomes in reconstructed epithelia. Gene Ther. Oct 1998;5(10):1322-32. [Medline].

  21. Mavilio F, Pellegrini G, Ferrari S, et al. Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cells. Nat Med. Dec 2006;12(12):1397-402. [Medline].

  22. Terrill PJ, Mayou BJ, Pemberton J. Experience in the surgical management of the hand in dystrophic epidermolysis bullosa. Br J Plast Surg. Aug-Sep 1992;45(6):435-42. [Medline].

  23. Ames WA, Mayou BJ, Williams KN. Anaesthetic management of epidermolysis bullosa. Br J Anaesth. May 1999;82(5):746-51. [Medline].

  24. Falabella AF, Valencia IC, Eaglstein WH, Schachner LA. Tissue-engineered skin (Apligraf) in the healing of patients with epidermolysis bullosa wounds. Arch Dermatol. Oct 2000;136(10):1225-30. [Medline].

  25. Cameli N, Picardo M, Pisani A, Ortonne JP, Tosti A. Characterization of the nail matrix basement membrane zone: an immunohistochemical study of normal nails and of the nails in Herlitz junctional epidemolysis bullosa. Br J Dermatol. Jan 1996;134(1):182-4. [Medline].

  26. Ciccarelli AO, Rothaus KO, Carter DM, Lin AN. Plastic and reconstructive surgery in epidermolysis bullosa: clinical experience with 110 procedures in 25 patients. Ann Plast Surg. Sep 1995;35(3):254-61. [Medline].

  27. Darling TN, Bauer JW, Hintner H, Yancey KB. Generalized atrophic benign epidermolysis bullosa. Adv Dermatol. 1997;13:87-119; discussion 120. [Medline].

  28. Fine JD, Bauer EA, Briggaman RA, et al. Revised clinical and laboratory criteria for subtypes of inherited epidermolysis bullosa. A consensus report by the Subcommittee on Diagnosis and Classification of the National Epidermolysis Bullosa Registry. J Am Acad Dermatol. Jan 1991;24(1):119-35. [Medline].

  29. Haynes L, Atherton D, Clayden G. Constipation in epidermolysis bullosa: successful treatment with a liquid fiber-containing formula. Pediatr Dermatol. Sep-Oct 1997;14(5):393-6. [Medline].

  30. Kerns ML, DePianto D, Dinkova-Kostova AT, Talalay P, Coulombe PA. Reprogramming of keratin biosynthesis by sulforaphane restores skin integrity in epidermolysis bullosa simplex. Proc Natl Acad Sci U S A. Sep 4 2007;104(36):14460-5. [Medline].

  31. Lansdown R, Atherton D, Dale A, Sproston S, Lloyd J. Practical and psychological problems for parents of children with epidermolysis bullosa. Child Care Health Dev. Jul-Aug 1986;12(4):251-6. [Medline].

  32. Lin AN, Carter DM. Epidermolysis bullosa. Annu Rev Med. 1993;44:189-99. [Medline].

  33. Marinkovich MP. Update on inherited bullous dermatoses. Dermatol Clin. Jul 1999;17(3):473-85, vii. [Medline].

  34. Marinkovich MP, Herron GS, Khavari PA, Bauer EA. Inheritied Epidermolysis Bullosa. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, Fitzpatrick TB, eds. Fitzpatrick's Dermatology in General Medicine. 6th ed. New York, NY: McGraw-Hill; 2003:596-609.

  35. Marinkovich MP, Verrando P, Keene DR, et al. Basement membrane proteins kalinin and nicein are structurally and immunologically identical. Lab Invest. Sep 1993;69(3):295-9. [Medline].

  36. McAllister JC, Peter Marinkovich M. Advances in inherited epidermolysis bullosa. Adv Dermatol. 2005;21:303-34. [Medline].

  37. McGrath JA, Eady RA. Molecular basis of blistering skin diseases. Hosp Med. Jan 1998;59(1):28-32. [Medline].

  38. McGrath JA, Ishida-Yamamoto A, Tidman MJ, Heagerty AH, Schofield OM, Eady RA. Epidermolysis bullosa simplex (Dowling-Meara). A clinicopathological review. Br J Dermatol. May 1992;126(5):421-30. [Medline].

  39. McGrath JA, Schofield OM, Mayou BJ, McKee PH, Eady RA. Epidermolysis bullosa complicated by squamous cell carcinoma: report of 10 cases. J Cutan Pathol. Apr 1992;19(2):116-23. [Medline].

  40. Mellerio JE, Weiner M, Denyer JE, et al. Medical management of epidermolysis bullosa: Proceedings of the IInd International Symposium on Epidermolysis Bullosa, Santiago, Chile, 2005. Int J Dermatol. Aug 2007;46(8):795-800. [Medline].

  41. Meneguzzi G, Marinkovich MP, Aberdam D, Pisani A, Burgeson R, Ortonne JP. Kalinin is abnormally expressed in epithelial basement membranes of Herlitz's junctional epidermolysis bullosa patients. Exp Dermatol. Dec 1992;1(5):221-9. [Medline].

  42. Olivry T, Dunston SM, Marinkovich MP. Reduced anchoring fibril formation and collagen VII immunoreactivity in feline dystrophic epidermolysis bullosa. Vet Pathol. Nov 1999;36(6):616-8. [Medline].

  43. Ortiz-Urda S, Lin Q, Green CL, Keene DR, Marinkovich MP, Khavari PA. Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue. J Clin Invest. Jan 2003;111(2):251-5. [Medline].

  44. Pfendner E, Rouan F, Uitto J. Progress in epidermolysis bullosa: the phenotypic spectrum of plectin mutations. Exp Dermatol. Apr 2005;14(4):241-9. [Medline].

  45. Powell AM, Sakuma-Oyama Y, Oyama N, Black MM. Collagen XVII/BP180: a collagenous transmembrane protein and component of the dermoepidermal anchoring complex. Clin Exp Dermatol. Nov 2005;30(6):682-7. [Medline].

  46. Ryan MC, Christiano AM, Engvall E, et al. The functions of laminins: lessons from in vivo studies. Matrix Biol. Dec 1996;15(6):369-81. [Medline].

  47. Schober-Flores C. Epidermolysis bullosa: a nursing perspective. Dermatol Nurs. Aug 1999;11(4):243-8, 253-6. [Medline].

  48. Seitz CS, Giudice GJ, Balding SD, Marinkovich MP, Khavari PA. BP180 gene delivery in junctional epidermolysis bullosa. Gene Ther. Jan 1999;6(1):42-7. [Medline].

  49. Shaw DW, Fine JD, Piacquadio DJ, Greenberg MJ, Wang-Rodriguez J, Eichenfield LF. Gastric outlet obstruction and epidermolysis bullosa. J Am Acad Dermatol. Feb 1997;36(2 Pt 2):304-10. [Medline].

  50. Tabas M, Gibbons S, Bauer EA. The mechanobullous diseases. Dermatol Clin. Jan 1987;5(1):123-36. [Medline].

  51. Terrill PJ, Mayou BJ, McKee PH, Eady RA. The surgical management of dystrophic epidermolysis bullosa (excluding the hand). Br J Plast Surg. Aug-Sep 1992;45(6):426-34. [Medline].

  52. Uitto J, Eady R, Fine JD, Feder M, Dart J. The DEBRA International Visioning/Consensus Meeting on Epidermolysis Bullosa: summary and recommendations. J Invest Dermatol. Apr 2000;114(4):734-7. [Medline].

  53. Uitto J, Pulkkinen L, McLean WH. Epidermolysis bullosa: a spectrum of clinical phenotypes explained by molecular heterogeneity. Mol Med Today. Oct 1997;3(10):457-65. [Medline].

  54. Vaisanen L, Has C, Franzke C, et al. Molecular mechanisms of junctional epidermolysis bullosa: Col 15 domain mutations decrease the thermal stability of collagen XVII. J Invest Dermatol. Dec 2005;125(6):1112-8. [Medline].

  55. Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent. Jul-Aug 1993;15(4):242-8. [Medline].

 
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Epidermolysis bullosa simplex, Weber-Cockayne subtype. This mild bullous disease is characterized by localized blistering at sites of trauma such as the feet.
Epidermolysis bullosa simplex, Koebner subtype. Palmoplantar blistering and hyperkeratosis are noted.
Epidermolysis bullosa simplex, Koebner subtype. Close-up image shows hyperkeratotic papules and plaques on the palm.
Junctional epidermolysis bullosa, Herlitz subtype. This severe disease is characterized by generalized intralamina lucida blistering at birth, significant internal involvement, and a poor prognosis.
Dominantly inherited dystrophic epidermolysis bullosa. The blistering in this disease often is localized and is characterized by scarring and milia in healed blister sites.
Dominantly inherited dystrophic epidermolysis bullosa. This subtype, similar to other dystrophic and junctional epidermolysis bullosa subtypes, can result in nail dystrophy and loss.
Recessively inherited dystrophic epidermolysis bullosa pseudosyndactyly (mitten-hand deformity) of the hands and feet
Recessively inherited dystrophic epidermolysis bullosa, oral cavity blistering and scarring
Recessively inherited dystrophic epidermolysis bullosa, squamous cell carcinoma
Diagram illustrating the organization of the dermal epidermal basement membrane and level of disruption in epidermolysis bullosa subtypes
 
 
 
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