eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Vulgaris: Differential Diagnoses & Workup

Author: Bassam Zeina, MD, PhD, Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK
Coauthor(s): Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK
Contributor Information and Disclosures

Updated: Aug 14, 2009

Differential Diagnoses

Bullous Pemphigoid
Pemphigus Herpetiformis
Dermatitis Herpetiformis
Pemphigus, Drug-Induced
Erythema Multiforme
Pemphigus, IgA
Familial Benign Pemphigus (Hailey-Hailey Disease)
Pemphigus, Paraneoplastic
Linear IgA Dermatosis
Pemphigus Erythematosus
Pemphigus Foliaceus

Other Problems to Be Considered

Erythema multiforme
Aphthous ulcers
Herpetic stomatitis
Bullous lichen planus

Workup

Laboratory Studies

  • To establish a diagnosis of pemphigus vulgaris (PV), perform the following tests:
    • Histopathology from the edge of a blister
    • Direct immunofluorescence (DIF) on normal-appearing perilesional skin
    • Indirect immunofluorescence (IDIF) using the patient's serum if DIF is positive. The preferred substrate for IDIF is monkey esophagus or salt-split normal human skin substrate.
  • DIF demonstrates in vivo deposits of antibodies and other immunoreactants, such as complement. DIF usually shows IgG deposited on the surface of the keratinocytes in and around lesions. IgG1 and IgG4 are the most common subclasses. Complement components such as C3 and immunoglobulin M are present less frequently than IgG. DIF shows intercellular deposition throughout the epidermis. This pattern of immunoreactants is not specific for PV and may be seen in pemphigus vegetans, pemphigus foliaceus, and pemphigus erythematosus. The best location for DIF is normal perilesional skin. When DIF is performed on lesional skin, false-positive results can be observed.

    Direct immunofluorescence showing intercellular i...

    Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

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    Direct immunofluorescence showing intercellular i...

    Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

  • Skin biopsy specimens placed in transport media may yield false-negative results; therefore, fresh tissue is the preferred substrate for DIF studies.
  • In the patient's serum, IDIF demonstrates the presence of circulating IgG autoantibodies that bind to epidermis. Circulating intercellular antibodies are detected using IDIF in 80-90% of patients with PV. The titer of circulating antibody correlates with disease course.20

Histologic Findings

Histopathology demonstrates an intradermal blister. The earliest changes consist of intercellular edema with loss of intercellular attachments in the basal layer. Suprabasal epidermal cells separate from the basal cells to form clefts and blisters. Basal cells are separated from one another and stand like a row of tombstones on the floor of the blister, but they remain attached to the basement membrane. Blister cells contain some acantholytic cells. Histopathology can help differentiate PV from pemphigus foliaceous, which demonstrates a more superficial epidermal cleavage.

Tzanck preparation is a smear taken from the base of a blister or an oral erosion that contains acantholytic cells. Blistering is preceded by eosinophilic spongiosis in some patients. The superficial dermis has a mild, superficial, mixed inflammatory infiltrate, which includes some eosinophils.

More on Pemphigus Vulgaris

Overview: Pemphigus Vulgaris
Differential Diagnoses & Workup: Pemphigus Vulgaris
Treatment & Medication: Pemphigus Vulgaris
Follow-up: Pemphigus Vulgaris
Multimedia: Pemphigus Vulgaris
References
Further Reading
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Keywords

pemphigus vulgaris, PV, bullous disease

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Contributor Information and Disclosures

Author

Bassam Zeina, MD, PhD, Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK
Bassam Zeina, MD, PhD is a member of the following medical societies: British Association of Dermatologists, British Medical Association, and Royal Society of Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK
Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Anti-Aging Medicine, American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians and Surgeons of Glasgow, and Royal College of Physicians of the United Kingdom
Disclosure: Nothing to disclose.

Medical Editor

Abby S Van Voorhees, MD, Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania
Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Genentech Honoraria Consulting; Incyte Grant/research funds Other; Warner Chilcott Honoraria Consulting; Merck Salary Management position; Abbott  Speaking and teaching

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

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

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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