eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigoid Gestationis: Differential Diagnoses & Workup

Author: Anatoli Freiman, MD, FRCPC, DABD, Consulting Staff, Division of Dermatology, Women's College Hospital, University of Toronto
Coauthor(s): Anju Pabby, MD, Consulting Staff, LK Dermatology and Laser Center
Contributor Information and Disclosures

Updated: Dec 9, 2008

Differential Diagnoses

Bullous Pemphigoid
Cicatricial Pemphigoid
Linear IgA Dermatosis
Pruritic Urticarial Papules and Plaques of Pregnancy
Urticaria, Acute

Other Problems to Be Considered

Allergic contact dermatitis
Dermatitis herpetiformis
Drug-induced bullous disorders
Erythema multiforme
Papular dermatitis of pregnancy
Prurigo gestationis of Besnier
Pruritic folliculitis of pregnancy

Workup

Laboratory Studies

  • Routine laboratory studies are not helpful in diagnosing pemphigoid gestationis.
    • The results with most hematologic studies are within normal limits, although peripheral eosinophilia is not uncommon and may correlate with disease severity.
    • Laboratory values that may be elevated include immunoglobulin levels, erythrocyte sedimentation rates, acute phase reactant levels, and antithyroid antibodies.
  • The criteria for the diagnosis of pemphigoid gestationis include an appropriate clinical presentation, histologic findings of a subepidermal blistering process (as described below), and direct immunofluorescence (DIF) results that show a linear band of C3 deposition with or without immunoglobulin G (present in 20-25% of patients) along the basement membrane.
    • The DIF test is the key assay to differentiate pemphigoid gestationis (positive DIF findings) from pruritic urticarial papules and plaques of pregnancy (negative DIF findings). However, a similar pattern of DIF results is observed in patients with pemphigoid gestationis, BP, and epidermolysis bullosa acquisita (EBA).
    • DIF should be performed using samples from noninvolved perilesional skin.
  • Indirect immunofluorescence (IDIF) testing of the patient's serum can be used to detect circulating antibodies for basement membrane zone. In 2004, Sitaru et al3 demonstrated that immunoblotting and enzyme-linked immunosorbent assay testing are sensitive tools for the detection of autoantibodies to BP180 antigen in patients with pemphigoid gestationis and that enzyme-linked immunosorbent assay is useful to monitor autoantibody serum levels.
  • HLA-DR3/DR4 is present in 45% of patients with pemphigoid gestationis, as compared with 3% of the general population.

Histologic Findings

Biopsy samples from the edge of an early blister typically reveal a subepidermal blister with an eosinophil-predominant infiltrate. The inflammatory infiltrate is localized to the dermal-epidermal junction and perivascular areas (see Media File 3). Keratinocyte necrosis and dermal edema are often present. These histologic features can also be observed in association with other autoimmune subepidermal blistering diseases, including BP and EBA (inflammatory type), cicatricial pemphigoid, and linear immunoglobulin A bullous dermatosis.

The salt-split technique demonstrates antibody deposition along the base of the epidermal fragment. IDIF reveals a similar localization in patients with BP, whereas patients with EBA have circulating autoantibodies that bind to the blister floor.

More on Pemphigoid Gestationis

Overview: Pemphigoid Gestationis
Differential Diagnoses & Workup: Pemphigoid Gestationis
Treatment & Medication: Pemphigoid Gestationis
Follow-up: Pemphigoid Gestationis
Multimedia: Pemphigoid Gestationis
References
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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. 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].

  14. 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].

  15. 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].

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

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Further Reading

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Keywords

herpes gestationis, PG, autoimmune dermatosis of pregnancy, pregnancy-associated autoimmune disease, bullous pemphigoid antigen 2, BPAG2, herpetiform blisters, herpes gestationis factor, pruritic urticarial papules, pruritic urticarial blisters

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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
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.

Medical Editor

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

Pharmacy Editor

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.

Managing Editor

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

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

 
 
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