bullous pemphigoid; parapemphigus

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Introduction

Autoimmune disorder characterized by a chronic vesiculobullous dermatitis. (Also see cicatricial pemphigoid)

Etiology

Epidemiology

  • two age groups: children & individuals 60-80 years of age
  • no sex preference
  • most common autoimmune bullous disease
    • up to 4.3 cases/100,000/year

Pathology

* histopathology images[18][20][21]

Genetics

Clinical manifestations

* images[18][22]

Laboratory

Complications

Differential diagnosis

Management

More general terms

Additional terms

References

  1. Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 406-409
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 166-67
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015. 2018
    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. 4.0 4.1 Beissert S et al, A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol 2007 Dec; 143:1536. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18087004
  5. 5.0 5.1 Parker SRS et al. Mortality of bullous pemphigoid: An evaluation of 223 patients and comparison with the mortality in the general population in the United States. J Am Acad Dermatol 2008 Oct; 59:582. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18707800
  6. 6.0 6.1 6.2 Bernard P et al Risk factors for relapse in patients with bullous pemphigoid in clinical remission: A multicenter, prospective, cohort study. Arch Dermatol 2009 May; 145:537. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19451497
  7. 7.0 7.1 7.2 7.3 7.4 Geriatric Review Syllabus, 7th edition Parada JT et al (eds) American Geriatrics Society, 2010
    Geriatric Review Syllabus, 8th edition (GRS8) Durso SC and Sullivan GN (eds) American Geriatrics Society, 2013
  8. 8.0 8.1 ARUP Consult: Pemphigoid The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/pemphigoid
  9. 9.0 9.1 Joly P et al. Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 2012 Aug; 132:1998. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22418872
    Naldi L et al Bullous pemphigoid: Simple measures for a complex disease. J Invest Dermatol 2012 Aug; 132:1948. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22797296
  10. 10.0 10.1 10.2 Bakker CV et al. Bullous pemphigoid as pruritus in the elderly. JAMA Dermatol 2013 Jun 26 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23804286 <Internet> http://archderm.jamanetwork.com/article.aspx?articleid=1699369
    Hertl M and Schmidt T. Underrecognition of the heterogeneous clinical spectrum of bullous pemphigoid. JAMA Dermatol 2013 Jun 26; <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23804145 <Internet> http://archderm.jamanetwork.com/article.aspx?articleid=1699364
  11. Parker SR, MacKelfresh J. Autoimmune blistering diseases in the elderly. Clin Dermatol. 2011 Jan-Feb;29(1):69-79 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21146735
  12. Kirtschig G, Middleton P, Bennett C et al Interventions for bullous pemphigoid. Cochrane Database Syst Rev. 2010 Oct 6;(10):CD002292 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20927731
  13. Di Zenzo G, Marazza G, Borradori L. Bullous pemphigoid: physiopathology, clinical features and management. Adv Dermatol. 2007;23:257-88. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18159905
  14. 14.0 14.1 Saw VP, Dart JK. Ocular mucous membrane pemphigoid: diagnosis and management strategies. Ocul Surf. 2008 Jul;6(3):128-42. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18781259
  15. Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Dermatol Clin. 2011 Jul;29(3):427-38, viii-ix. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21605808
  16. 16.0 16.1 Cho Y-T et al. First-line combination therapy with rituximab and corticosteroids provides a high complete remission rate in moderate-to-severe bullous pemphigoid. Br J Dermatol 2014 Dec 22; <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25529394 <Internet> http://onlinelibrary.wiley.com/doi/10.1111/bjd.13633/abstract
  17. 17.0 17.1 Cai SCS et al. Association of bullous pemphigoid and malignant neoplasms. JAMA Dermatol 2015 Feb 11 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25671805 <Internet> http://archderm.jamanetwork.com/article.aspx?articleid=2110015
  18. 18.0 18.1 18.2 Raap U, Werfel T (images) IMAGES IN CLINICAL MEDICINE. Bullous Pemphigoid. N Engl J Med. 2015 Oct 22;373(17):1659. http://www.nejm.org/doi/full/10.1056/NEJMicm1500430 PMID: https://www.ncbi.nlm.nih.gov/pubmed/26488696
  19. Ruocco E, Wolf R, Caccavale S et al Bullous pemphigoid: associations and management guidelines: facts and controversies. Clin Dermatol. 2013 Jul-Aug;31(4):400-12 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23806157
  20. 20.0 20.1 Chan LS, Elston DM (histopathology images) Medscape: Bullous Pemphigoid http://emedicine.medscape.com/article/1062391-overview
  21. 21.0 21.1 DermNet NZ. (histopathology images) Bullous pemphigoid pathology http://www.dermnetnz.org/pathology/bp-path.html
  22. 22.0 22.1 DermNet. Skin Disease Atlas (images) Bullous Pemphigoid Photos http://www.dermnet.com/images/Bullous-Pemphigoid
  23. Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Immunol Allergy Clin North Am. 2012 May;32(2):217-32, v. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22560135
  24. 24.0 24.1 Shen WC, Chiang HY, Chen PS et al Risk of All-Cause Mortality, Cardiovascular Disease Mortality, and Cancer Mortality in Patients With Bullous Pemphigoid. JAMA Dermatol. Published online December 29, 2021. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34964804 https://jamanetwork.com/journals/jamadermatology/fullarticle/2787674
  25. NEJM Knowledge+ Dermatology
  26. 26.0 26.1 Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018;66:255-70. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29479654

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