Pemphigus Vulgaris Clinical Presentation

  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 28, 2011
 

History

  • Mucous membranes: Pemphigus vulgaris presents with oral lesions in 50-70% of patients, and almost all patients have mucosal lesions at some point in the course of their disease. Mucosal lesions may be the sole sign for an average of 5 months before skin lesions develop, or they may be the sole manifestation of the disease. The diagnosis of pemphigus vulgaris should be considered in any patient with persistent oral erosive lesions.
  • Skin: Most patients with pemphigus vulgaris develop cutaneous lesions. The primary lesion of pemphigus vulgaris is a flaccid blister, which usually arises on healthy-appearing skin but may be found on erythematous skin. New blisters usually are flaccid or become flaccid quickly. Affected skin often is painful but rarely pruritic.
  • Drug-induced pemphigus vulgaris[11] : Drugs reported most significantly in association with pemphigus vulgaris include penicillamine, captopril, cephalosporin, pyrazolones, nonsteroidal anti-inflammatory drugs (NSAIDs), and other thiol-containing compounds. Rifampin, emotional stress, thermal burns, ultraviolet rays, and infections (eg, coxsackievirus, Herpesviridae family) have also been reported as triggers for pemphigus vulgaris.[12]
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Physical

Mucous membranes typically are affected first in pemphigus vulgaris. Mucosal lesions may precede cutaneous lesions by weeks or months. Patients with mucosal lesions may present to dentists, oral surgeons, or gynecologists.[13]

  • Mucous membranes
    • Intact bullae are rare in the mouth. More commonly, patients have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. The erosions extend peripherally with shedding of the epithelium.
    • The mucous membranes most often affected in pemphigus vulgaris are those of the oral cavity, which is involved in almost all patients with pemphigus vulgaris and sometimes is the only area involved. Erosions may be seen on any part of the oral cavity. Erosions can be scattered and often are extensive. Erosions may spread to involve the larynx, with subsequent hoarseness. The patient often is unable to eat or drink adequately because the erosions are so uncomfortable.
    • In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of the cases.
    • Other mucosal surfaces may be involved, including the conjunctiva,[14] esophagus (causes odynophagia and/or dysphagia),[15] labia, vagina, cervix, vulva,[16] penis, urethra, nasal mucosa, and anus.
  • Skin
    • The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base, as shown in the images below. Early, small blister filled with clear fluid ariseEarly, small blister filled with clear fluid arises on healthy skin. Flaccid blister filled with clear fluid arises on Flaccid blister filled with clear fluid arises on healthy skin.
    • The blisters are fragile; therefore, intact blisters may be sparse. The contents soon become turbid, or the blisters rupture, producing painful erosions, which is the most common skin presentation and is shown in the image below. Erosions often are large because of their tendency to extend peripherally with the shedding of the epithelium. An erosion. An erosion.
  • Vegetating pemphigus vulgaris: Ordinary pemphigus vulgaris erosions may develop vegetation. Lesions in skin folds readily form vegetating granulations. In some patients, erosions tend to develop excessive granulation tissue and crusting, and these patients display more vegetating lesions. This type of lesion tends to occur more frequently in intertriginous areas and on the scalp or face. The vegetating type of response can be more resistant to therapy and can remain in one place for long periods.
  • Nails: Acute or chronic paronychia, subungual hematomas, and nail dystrophies affecting one or several fingers or toes have been reported with pemphigus vulgaris.[17, 18] Patients with paronychial pemphigus usually also have oral involvement.
  • Pemphigus in pregnancy: Pemphigus vulgaris occurring in pregnancy is rare. When present, maternal autoantibodies may cross the placenta, resulting in neonatal pemphigus. Neonatal pemphigus is transient and improves with clearance of maternal autoantibodies.[19] Treatment of pemphigus vulgaris in pregnancy is with oral corticosteroids; however, prednisone and its metabolites cross the placenta and have been associated with low birth weight, prematurity, infection, and adrenal insufficiency.
  • Nikolsky sign: In patients with active blistering, firm sliding pressure with a finger separates normal-appearing epidermis, producing an erosion. This sign is not specific for pemphigus vulgaris and is found in other active blistering diseases.
  • Asboe-Hansen sign: Lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin.
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Causes

The cause of pemphigus vulgaris remains unknown; however, several potentially relevant factors have been identified.

  • Genetic factors: Predisposition to pemphigus is linked to genetic factors.[20] Certain major histocompatibility complex (MHC) class II molecules, in particular alleles of human leukocyte antigen DR4 (DRB1*0402) and human leukocyte antigen DRw6 (DQB1*0503), are common in patients with pemphigus vulgaris.[21, 22, 23, 24, 25, 26]
  • Age: Peak age of onset is from 50-60 years. Infants with neonatal pemphigus remit with clearance of maternal autoantibodies. The disease may develop in children or in older persons.
  • Disease association: Pemphigus occurs in patients with other autoimmune diseases, particularly myasthenia gravis and thymoma.[27] A study of 110 patients with pemphigus found 4 patients with autoimmune thyroid disease and 3 patients with rheumatoid arthritis. In this study, however, autoimmune diseases were no more common in 969 first-degree relatives of patients with pemphigus than in the general population.[28]
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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)

Nicole Sakka, MBBS  Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK

Disclosure: Nothing to disclose.

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.

Specialty Editor Board

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; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

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.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), 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 Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

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.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Mohsin Ali, MBBS, FRCP, MRCP, to the development and writing of this article.

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Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on healthy skin.
An erosion.
Erosions and healing areas on the back.
Healing areas on the chest and abdomen.
Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.
 
 
 
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