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Pemphigoid Gestationis

  • Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Aug 17, 2015
 

Background

Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis of pregnancy (see the image below). 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.

Tense bullae are present on the arms of this other Tense bullae are present on the arms of this otherwise healthy 32-year-old primigravida woman.

See Diagnosing Dermatoses in Pregnant Patients: 8 Cases to Test Your Skills, a Critical Images slideshow, for help identifying several types of cutaneous eruptions associated with pregnancy.

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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.

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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.

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.

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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

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, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Board of Dermatology<br/>Co-Editor for the text Dermatological Manifestations of Kidney Disease .

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

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

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

Disclosure: Received consulting fee from Novan for consulting; Received consulting fee from Stieffel, a GSK company for consulting; Received salary from Society for Investigative Dermatology for board membership.

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. 1999 Jul. 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. 2006 Mar. 54(3):395-404. [Medline].

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

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

  8. 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].

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

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

 
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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.
 
 
 
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