Pemphigoid Gestationis Clinical Presentation

  • Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Feb 28, 2012
 

History

Pemphigoid gestationis typically manifests during late pregnancy, with an abrupt onset of extremely pruritic urticarial papules and blisters on the abdomen and trunk. Unrelenting pruritus often interferes with daily activities.

Lesions may appear any time during pregnancy, but they most commonly develop during the second and third trimesters.

Symptoms may abate at the end of pregnancy; however, dramatic flares can occur at or immediately after delivery. Pemphigoid gestationis usually resolves spontaneously within weeks to months after delivery and possibly quicker with breastfeeding. The persistence of disease activity for years postpartum has been reported.

Pemphigoid gestationis may recur with the resumption of menses, use of oral contraception, and subsequent pregnancies. The 1999 cohort study by Jenkins et al[2] showed no association between change in partner and development of pemphigoid gestationis in subsequent pregnancies.

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Physical

The initial clinical manifestations are erythematous urticarial patches and plaques, which are typically periumbilical. These lesions progress to tense vesicles and blisters. Some patients may present with urticarial plaques and may never develop blisters (see the images below). These hivelike plaques differ from true urticaria because of their relatively fixed nature. The rash spreads peripherally, often sparing the face, palms, and soles. Mucosal lesions occur in less than 20% of cases. Patients may have secondary infections at blister sites.

Tense bullae are present on the arms of this otherTense bullae are present on the arms of this otherwise healthy 32-year-old primigravida woman. Urticarial or hivelike plaques, as seen on the posUrticarial or hivelike plaques, as seen on the posterolateral neck of this woman in her third trimester, can be observed in patients with pemphigoid gestationis. A close-up view of a blister reveals the tense priA close-up view of a blister reveals the tense primary lesion filled with clear fluid.
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Causes

Pemphigoid gestationis is a pregnancy-associated autoimmune disease. The autoantibodies are deposited in the skin and detectable in the circulation, and they are predominantly specific for the hemidesmosomal protein BPAG2.

Circulating antibodies and T cells are directed against an immunodominant epitope. This epitope, located in the extracellular region of BPAG2 near the membrane, is called the MCW-1 domain. This region of BPAG2 is also an immunodominant epitope in a closely related autoimmune blistering disease, BP.

The trigger for autoantibody production is still poorly understood. As described in Pathophysiology, autoantibodies to amniotic basement membrane (paternal major histocompatibility class II antigens) may cross-react with BPAG2 antigen in the skin, leading to the immune response.

Pemphigoid gestationis has also been described to occur in association with trophoblastic tumors, such as hydatiform mole or choriocarcinoma.

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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 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  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 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; Vernallis Honoraria Consulting

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, 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: 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, Ackerman Academy of Dermatopathology, New York

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].

 
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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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