Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Epidermolysis Bullosa Acquisita Treatment & Management

  • Author: Daniel L Croom, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Apr 29, 2016
 

Medical Care

Patients with epidermolysis bullosa acquisita (EBA) may require therapy with oral corticosteroids, anti-inflammatory agents, and immunosuppressants.[26, 27, 28, 29, 30]

For patients who are on long-term systemic corticosteroid treatment, daily calcium, vitamin D, and potentially bisphosphonate therapy are important for reducing steroid-induced osteoporosis.

Next

Consultations

Coordination of care with the patient's primary care provider is important for monitoring adverse effects of therapy and the overall health of the patient.

Because epidermolysis bullosa acquisita (EBA) can involve esophageal mucosa and is strongly associated with inflammatory bowel disease, patients with epidermolysis bullosa acquisita should be asked about symptoms related to their GI tract. If there are any symptoms suggestive of esophageal lesions or inflammatory bowel disease, patients with epidermolysis bullosa acquisita should be referred to a gastroenterologist.

In patients with oral involvement, coordination of care with the patient's dentist is recommended.

In patients with ocular involvement, consultation with an ophthalmologist is recommended.

Previous
Next

Diet

In patients with oral involvement, hard or brittle foods and foods with high acid content (eg, tomatoes, orange juice) should be avoided. Ingestion of these foods may traumatize mucosa and precipitate new lesions.

Previous
Next

Activity

Because epidermolysis bullosa acquisita (EBA) primarily affects trauma-prone skin surfaces, patients are instructed to avoid direct physical trauma to their skin surfaces. When physical activities are planned, patients are instructed to use protective pads to cover their extensor skin surfaces. Additionally, gentle but thorough daily oral hygiene should be encouraged.

Previous
Next

Complications

Infection is a possible consequence of open erosions and wounds in the setting of immunosuppression.

Malignancies may occur, secondary to chronic inflammation and immunosuppressive treatments.

Bone marrow suppression is possible, secondary to medication.

Growth retardation may occur, secondary to medications used during childhood.

Complications secondary to prednisone treatment for epidermolysis bullosa acquisita (EBA) include adrenal insufficiency, osteoporosis, and cataracts.

The chronic inflammation and scar formation on patients' extensor surfaces can severely hinder the daily activities of patients.[31] The scarring nature of epidermolysis bullosa acquisita can lead to nail destruction and hair loss.

Oropharyngeal mucous membrane involvement can lead to periodontal disease, oral mucosal erosions, and esophageal strictures, which limit oral intake, and supraglottic stenosis with airway compromise. Ocular involvement can lead to obstruction of nasolacrimal ducts, conjunctival scarring, and blindness.

Previous
Next

Prevention

It is important to monitor and prevent complications of prolonged systemic corticosteroid therapy. The American College of Rheumatology 2010 guidelines for prevention and treatment of glucocorticoid-induced osteoporosis recommend calcium and vitamin D supplementation, lifestyle modification counseling, and consideration of bisphosphonate therapy.[32] The indication for bisphosphonate therapy is based on risk stratification considering age, steroid dose, duration of treatment, and Fracture Risk Assessment Tool (FRAX) score. Prolonged corticosteroid treatment is also a risk factor for cataracts, and patients should be screened by an ophthalmologist.

Previous
Next

Long-Term Monitoring

Patients with epidermolysis bullosa acquisita (EBA) should be monitored regularly by physicians with experience in treating autoimmune skin disease. During the active disease stage, patients should be monitored by their physicians on a monthly basis. When in remission, patients should be monitored by their physicians annually.

Previous
 
 
Contributor Information and Disclosures
Author

Daniel L Croom, MD Undersea Medical Officer, Naval Special Warfare Center

Disclosure: Nothing to disclose.

Coauthor(s)

Kristina Marie Dela Rosa, MD Dermatologist, Naval Hospital Camp Pendleton

Kristina Marie Dela Rosa, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Jaggi Rao, MD, FRCPC Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, Director of Dermatology Residency Program, University of Alberta Faculty of Medicine and Dentistry

Jaggi Rao, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Medical Association, Pacific Dermatologic Association, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Ponciano D Cruz, Jr, MD Professor and Vice-Chair, Paul R Bergstresser Chair, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz, Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: Received consulting fee from RCTS for independent contractor; Received honoraria from Mary Kay Cosmetics for consulting; Received grant/research funds from Galderma for principal investigator.

Acknowledgements

Lawrence S Chan, MD Dr Orville J Stone Professor of Dermatology, Head, Department of Dermatology, University of Illinois College of Medicine

Lawrence S Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Dermatological Association, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, Microcirculatory Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

David Woodley, MD Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California

David Woodley, MD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association

Disclosure: Nothing to disclose.

The view(s) expressed herein are those of the authors and do not reflect the official policy or position of Naval Special Warfare Center, the U.S. Navy Medical Department, the U.S. Navy Office of the Surgeon General, the Department of the Navy, Department of Defense, or the U.S. Government.

References
  1. Olivry T, Fine J, Dunston SM, et al. Canine epidermolysis bullosa acquisita: Circulating autoantibodies target the aminoterminal noncollagenous (NC1) domain of collagen VII in anchoring fibrils. Vet Dermatol. 1998. 9:19-32.

  2. Xu L, Chen M, Peng J, O'Toole EA, Woodley DT, Chan LS. Molecular cloning and characterization of a cDNA encoding canine type VII collagen non-collagenous (NC1) domain, the target antigen of autoimmune disease epidermolysis bullosa acquisita (EBA). Biochim Biophys Acta. 1998 Oct 22. 1408(1):25-34. [Medline].

  3. Chen M, Keene DR, Costa FK, Tahk SH, Woodley DT. The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysis Bullosa acquisita autoantibodies. J Biol Chem. 2001 Jun 15. 276 (24):21649-55. [Medline].

  4. Gandhi K, Chen M, Aasi S, Lapiere JC, Woodley DT, Chan LS. Autoantibodies to type VII collagen have heterogeneous subclass and light chain compositions and their complement-activating capacities do not correlate with the inflammatory clinical phenotype. J Clin Immunol. 2000 Nov. 20(6):416-23. [Medline].

  5. Sitaru C, Mihai S, Otto C, et al. Induction of dermal-epidermal separation in mice by passive transfer of antibodies specific to type VII collagen. J Clin Invest. 2005 Apr. 115(4):870-8. [Medline].

  6. Woodley DT, Ram R, Doostan A, et al. Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients. J Invest Dermatol. 2006 Jun. 126(6):1323-30. [Medline].

  7. Chen L, Peterson JD, Zheng WY, Lin SX, Chan LS. Autoimmunity to type VII collagen in SKH1 mice is independent of regulatory T cells. Clin Exp Immunol. 2006 Aug. 145(2):322-31. [Medline].

  8. Ludwig R. Immune mechanism-targeted treatment of experimental epidermolysis bullosa acquisita. Expert Rev Clin Immunol. 2015 Dec. 11 (12):1365-78. [Medline].

  9. Ellebrecht CT, Srinivas G, Bieber K, Banczyk D, Kalies K, Künzel S, et al. Skin microbiota-associated inflammation precedes autoantibody induced tissue damage in experimental epidermolysis bullosa acquisita. J Autoimmun. 2015 Sep 1. [Medline].

  10. Muller R, Dahler C, Mobs C, et al. T and B cells target identical regions of the non-collagenous domain 1 of type VII collagen in epidermolysis bullosa acquisita. Clin Immunol. Jan/2010. EPub:[Medline].

  11. Peterson JD, Chan LS. Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the initial use to 2007. Ther Clin Risk Manag. Feb/2009. 5:1-7. [Medline]. [Full Text].

  12. Saha M, Cutler T, Bhogal B, Black MM, Groves RW. Refractory epidermolysis bullosa acquisita: successful treatment with rituximab. Clin Exp Dermatol. Dec/2009. 34:e979-80. [Medline].

  13. Iranzo P, Herrero-González JE, Mascaró-Galy JM, Suárez-Fernández R, España A. Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain. Br J Dermatol. 2014 Nov. 171 (5):1022-30. [Medline].

  14. Hashimoto T, Ishiko A, Shimizu H, et al. A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies. Br J Dermatol. 1996 Feb. 134(2):336-9. [Medline].

  15. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002 Mar. 138(3):370-9. [Medline].

  16. Derrow AE, Mutasim DF. Periorbital papulovesicular eruption in an elderly man. Localized periorbital epidermolysis bullosa acquisita (EBA). Arch Dermatol. 2009 May. 145(5):589-94. [Medline].

  17. Zumelzu C, Le Roux-Villet C, Loiseau P, et al. Black Patients of African Descent and HLA-DRB1*15:03 Frequency Overrepresented in Epidermolysis Bullosa Acquisita. J Invest Dermatol. 2011 Dec. 131(12):2386-93. [Medline].

  18. Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol. 1998 Feb. 110(2):103-9. [Medline].

  19. Chen M, Marinkovich MP, Jones JC, O'Toole EA, Li YY, Woodley DT. NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats. J Invest Dermatol. 1999 Feb. 112(2):177-83. [Medline].

  20. Jonkman MF, Schuur J, Dijk F, et al. Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha3. Arch Dermatol. 2000 Feb. 136(2):227-31. [Medline].

  21. Elston DM, Stratman EJ, Miller SJ. Skin biopsy: Biopsy issues in specific diseases. J Am Acad Dermatol. 2016 Jan. 74 (1):1-16; quiz 17-8. [Medline].

  22. Chan LS, Fine JD, Briggaman RA, et al. Identification and partial characterization of a novel 105-kDalton lower lamina lucida autoantigen associated with a novel immune-mediated subepidermal blistering disease. J Invest Dermatol. 1993 Sep. 101(3):262-7. [Medline].

  23. Gammon WR, Briggaman RA, Inman AO 3rd, Queen LL, Wheeler CE. Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol. 1984 Feb. 82(2):139-44. [Medline].

  24. Chen M, Chan LS, Cai X, O'Toole EA, Sample JC, Woodley DT. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol. 1997 Jan. 108(1):68-72. [Medline].

  25. Lehman JS, Camilleri MJ, Gibson LE. Epidermolysis bullosa acquisita: concise review and practical considerations. Int J Dermatol. 2009 Mar. 48 (3):227-35; quiz 235-6. [Medline].

  26. Crichlow SM, Mortimer NJ, Harman KE. A successful therapeutic trial of rituximab in the treatment of a patient with recalcitrant, high-titre epidermolysis bullosa acquisita. Br J Dermatol. 2007 Jan. 156(1):194-6. [Medline].

  27. Niedermeier A, Eming R, Pfütze M, et al. Clinical response of severe mechanobullous epidermolysis bullosa acquisita to combined treatment with immunoadsorption and rituximab (anti-CD20 monoclonal antibodies). Arch Dermatol. 2007 Feb. 143(2):192-8. [Medline].

  28. Sadler E, Schafleitner B, Lanschuetzer C, et al. Treatment-resistant classical epidermolysis bullosa acquisita responding to rituximab. Br J Dermatol. 2007 Aug. 157(2):417-9. [Medline].

  29. Schmidt E, Benoit S, Brocker EB, Zillikens D, Goebeler M. Successful adjuvant treatment of recalcitrant epidermolysis bullosa acquisita with anti-CD20 antibody rituximab. Arch Dermatol. 2006 Feb. 142(2):147-50. [Medline].

  30. Wallet-Faber N, Franck N, Batteux F, et al. Epidermolysis bullosa acquisita following bullous pemphigoid, successfully treated with the anti-CD20 monoclonal antibody rituximab. Dermatology. 2007. 215(3):252-5. [Medline].

  31. Iwata H, Witte M, Samavedam UK, Gupta Y, Shimizu A, Ishiko A, et al. Radiosensitive Hematopoietic Cells Determine the Extent of Skin Inflammation in Experimental Epidermolysis Bullosa Acquisita. J Immunol. 2015 Sep 1. 195 (5):1945-54. [Medline].

  32. Grossman JM, Gordon R, Ranganath VK, Deal C, Caplan L, Chen W, et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken). 2010 Nov. 62 (11):1515-26. [Medline].

  33. Intong LR, Murrell DF. Management of epidermolysis bullosa acquisita. Dermatol Clin. 2011 Oct. 29 (4):643-7. [Medline].

  34. Kim JH, Kim YH, Kim SC. Epidermolysis bullosa acquisita: a retrospective clinical analysis of 30 cases. Acta Derm Venereol. 2011 May. 91 (3):307-12. [Medline].

  35. Gürcan HM, Ahmed AR. Current concepts in the treatment of epidermolysis bullosa acquisita. Expert Opin Pharmacother. 2011 Jun. 12 (8):1259-68. [Medline].

  36. Ahmed AR, Gürcan HM. Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non-responsive to conventional therapy: clinical outcome and post-treatment long-term follow-up. J Eur Acad Dermatol Venereol. 2012 Sep. 26 (9):1074-83. [Medline].

 
Previous
Next
 
Direct immunofluorescence performed on perilesional skin biopsy specimen from a patient with epidermolysis bullosa acquisita detects a linear band of immunoglobulin G deposit along the dermoepidermal junction.
Indirect immunofluorescence performed on salt-split normal human skin substrate using serum from a patient affected with epidermolysis bullosa acquisita detects immunoglobulin G class circulating autoantibodies that bind to the dermal (base) side of the basement membrane.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.