Pemphigoid Gestationis 

  • Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 18, 2010
 

Background

Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis of pregnancy. The disease was originally named herpes gestationis on the basis of the morphological herpetiform feature of the blisters, but this term is a misnomer because pemphigoid gestationis is not related to or associated with any active or prior herpes virus infection.

Next

Pathophysiology

Pemphigoid gestationis is a pregnancy-associated autoimmune disease. Most patients develop antibodies against 2 hemidesmosomal proteins, BP180 (BPAG2, collagen XVII) and less frequently BP230. Historically known as herpes gestationis factor, these circulating antibodies belong to the heat-stable immunoglobulin G1 subclass. The binding of immunoglobulin G to the basement membrane triggers an immune response, leading to the formation of subepidermal vesicles and blisters. In 1999, Chimanovitch et al[1] demonstrated that pemphigoid gestationis sera recognize 5 distinct epitopes within BP180 NC16A, 4 of which have been reported as major antigenic sites targeted by bullous pemphigoid antibodies.

The trigger for the development of autoantibodies in persons with pemphigoid gestationis remains elusive. Cross-reactivity between placental tissue and skin has been proposed to play a role. Pemphigoid gestationis has a strong association with HLA-DR3 (61-80%) and HLA-DR4 (52%), or both (43-50%), and virtually all patients with a history of pemphigoid gestationis have demonstrable anti-HLA antibodies. The placenta is known to be the main source of disparate (paternal) antibodies and can thus present an immunologic target during gestation.

Previous
Next

Epidemiology

Frequency

United States

In the United States, pemphigoid gestationis has an estimated prevalence of 1 case in 50,000-60,000 pregnancies.

International

Findings from European studies suggest that pemphigoid gestationis has an overall incidence of 0.5 cases per million people per year. In 1999, Jenkins et al[2] described the largest cohort of 87 patients in the United Kingdom with a total of 278 pregnancies, of which 142 were complicated by pemphigoid gestationis.

Mortality/Morbidity

No increase in fetal or maternal mortality has been demonstrated. A greater prevalence of premature and small-for-gestational-age (SGA) babies is associated with pemphigoid gestationis. Of infants, 5-10% born to affected mothers may present with transient cutaneous involvement that resolves as maternal autoantibodies are cleared.

Patients with pemphigoid gestationis have a higher relative prevalence of other autoimmune diseases, including Hashimoto thyroiditis, Graves disease, and pernicious anemia, which are also associated with HLA-DR3 and DR-4 haplotypes

Race

Pemphigoid gestationis is less common among blacks than whites, which might reflect its association with specific HLA haplotypes.

Sex

This condition only affects females.

Age

Pemphigoid gestationis occurs in women of childbearing age.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Anatoli Freiman, MD, FRCPC, DABD  Consulting Staff, Division of Dermatology, Women's College Hospital, University of Toronto

Anatoli Freiman, MD, FRCPC, DABD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Anju Pabby, MD  Consulting Staff, LK Dermatology and Laser Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Russell Hall, MD  Chief, Professor, Department of Internal Medicine, Division of Dermatology, Duke University

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology

Disclosure: Genetech Grant/research funds Principle Investigator; Centecor Grant/research funds Principle Investigator

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Julia R Nunley, MD  Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex sub-investigator

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. Chimanovitch I, Schmidt E, Messer G, Dopp R, Partscht K, Brocker EB, et al. IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. J Invest Dermatol. Jul 1999;113(1):140-2. [Medline].

  2. Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol. Jul 1999;24(4):255-9. [Medline].

  3. Sitaru C, Powell J, Messer G, Brocker EB, Wojnarowska F, Zillikens D. Immunoblotting and enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. Obstet Gynecol. Apr 2004;103(4):757-63. [Medline].

  4. Amato L, Mei S, Gallerani I, Moretti S, Fabbri P. A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid?. J Am Acad Dermatol. Aug 2003;49(2):302-7. [Medline].

  5. Ambros-Rudolph CM, Mullegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol. Mar 2006;54(3):395-404. [Medline].

  6. Bedocs PM, Kumar V, Mahon MJ. Pemphigoid gestationis: a rare case and review. Arch Gynecol Obstet. May 28 2008;[Medline].

  7. Boulinguez S, Bedane C, Prost C, Bernard P, Labbe L, Bonnetblanc JM. Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. Dermatology. 2003;206(2):113-9. [Medline].

  8. Fabbri P, Caproni M, Berti S, Bianchi B, Amato L, De Pita O, et al. The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis. Br J Dermatol. Jun 2003;148(6):1141-8. [Medline].

  9. Kreuter A, Harati A, Breuckmann F, Appelhans C, Altmeyer P. Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. J Am Acad Dermatol. Dec 2004;51(6):1027-8. [Medline].

  10. Kroumpouzos G, Cohen LM. Dermatoses of pregnancy. J Am Acad Dermatol. Jul 2001;45(1):1-19; quiz 19-22. [Medline].

  11. Lin MS, Arteaga LA, Diaz LA. Herpes gestationis. Clin Dermatol. Nov-Dec 2001;19(6):697-702. [Medline].

  12. Lin MS, Gharia MA, Swartz SJ, Diaz LA, Giudice GJ. Identification and characterization of epitopes recognized by T lymphocytes and autoantibodies from patients with herpes gestationis. J Immunol. Apr 15 1999;162(8):4991-7. [Medline].

  13. Nair M, Kinagi R, Hickling DJ. Pemphigoid gestationis. J Obstet Gynaecol. Oct 2006;26(7):688-9. [Medline].

  14. Powell AM, Sakuma-Oyama Y, Oyama N, Albert S, Bhogal B, Kaneko F, et al. Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy. Arch Dermatol. 2005;141:705-10. [Medline].

  15. Satoh S, Seishima M, Sawada Y, Izumi T, Yoneda K, Kitajima Y. The time course of the change in antibody titres in herpes gestationis. Br J Dermatol. Jan 1999;140(1):119-23. [Medline].

  16. Semkova K, Black M. Pemphigoid gestationis: current insights into pathogenesis and treatment. Eur J Obstet Gynecol Reprod Biol. Aug 2009;145 (2):138-44. [Medline].

  17. Shimanovich I, Brocker EB, Zillikens D. Pemphigoid gestationis: new insights into the pathogenesis lead to novel diagnostic tools. BJOG. Sep 2002;109(9):970-6. [Medline].

  18. Tunzi M, Gray GR. Common skin conditions during pregnancy. Am Fam Physician. Jan 15 2007;75(2):211-8. [Medline].

 
Previous
Next
 
Tense bullae are present on the arms of this otherwise healthy 32-year-old primigravida woman.
Urticarial or hivelike plaques, as seen on the posterolateral neck of this woman in her third trimester, can be observed in patients with pemphigoid gestationis.
Upon histologic evaluation, an incipient blister is present at the junction of the epidermis and dermis, as is a moderately dense perivascular inflammatory infiltrate.
A close-up view of a blister reveals the tense primary lesion filled with clear fluid.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.