Pemphigus vulgaris

Jump to navigation Jump to search

WikiDoc Resources for Pemphigus vulgaris


Most recent articles on Pemphigus vulgaris

Most cited articles on Pemphigus vulgaris

Review articles on Pemphigus vulgaris

Articles on Pemphigus vulgaris in N Eng J Med, Lancet, BMJ


Powerpoint slides on Pemphigus vulgaris

Images of Pemphigus vulgaris

Photos of Pemphigus vulgaris

Podcasts & MP3s on Pemphigus vulgaris

Videos on Pemphigus vulgaris

Evidence Based Medicine

Cochrane Collaboration on Pemphigus vulgaris

Bandolier on Pemphigus vulgaris

TRIP on Pemphigus vulgaris

Clinical Trials

Ongoing Trials on Pemphigus vulgaris at Clinical

Trial results on Pemphigus vulgaris

Clinical Trials on Pemphigus vulgaris at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Pemphigus vulgaris

NICE Guidance on Pemphigus vulgaris


FDA on Pemphigus vulgaris

CDC on Pemphigus vulgaris


Books on Pemphigus vulgaris


Pemphigus vulgaris in the news

Be alerted to news on Pemphigus vulgaris

News trends on Pemphigus vulgaris


Blogs on Pemphigus vulgaris


Definitions of Pemphigus vulgaris

Patient Resources / Community

Patient resources on Pemphigus vulgaris

Discussion groups on Pemphigus vulgaris

Patient Handouts on Pemphigus vulgaris

Directions to Hospitals Treating Pemphigus vulgaris

Risk calculators and risk factors for Pemphigus vulgaris

Healthcare Provider Resources

Symptoms of Pemphigus vulgaris

Causes & Risk Factors for Pemphigus vulgaris

Diagnostic studies for Pemphigus vulgaris

Treatment of Pemphigus vulgaris

Continuing Medical Education (CME)

CME Programs on Pemphigus vulgaris


Pemphigus vulgaris en Espanol

Pemphigus vulgaris en Francais


Pemphigus vulgaris in the Marketplace

Patents on Pemphigus vulgaris

Experimental / Informatics

List of terms related to Pemphigus vulgaris

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and keywords:


Pemphigus vulgaris is a chronic blistering skin disease with skin lesions that are rarely pruritic, but which are often painful.[1]:561

Historical Perspective



It is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 resulting in the loss of cohesion between keratinocytes in the epidermis, classified as a type II hypersensitivity reaction (in which antibodies bind to antigens on the body's own tissues). It is characterized by extensive flaccid blisters and mucocutaneous erosions. The severity of the disease, as well as the mucosal lesions, is believed to be directly proportional to the levels of desmoglein 3. Milder forms of pemphigus (like foliacious and erythematoses) are more desmoglein 1 heavy. It arises most often in middle-aged or older people, usually starting with a blister that ruptures easily. The lesions can become quite extensive. The pathogenesis of the disease involves autoantibodies against desmosome proteins, separating keratinocytes from the basal layer of the epidermis. On histology, the basal keratinocytes are usually still attached to the basement membrane leading to the appearance and thus the term, "tombstoning".

Transudative fluid accumulates in between the keratinocytes and basement membrane (suprabasal split), forming a blister and resulting in what is known as a positive Nikolsky's sign. This is a contrasting feature from bullous pemphigoid, where the detachment occurs between the epidermis and dermis (subepidermal bullae).


Differentiating Pemphigus Vulgaris from other Diseases

Pemphigus vulgaris is easy to confuse with impetigo and candidiasis. IgG4 is considered pathogenic. The diagnosis can be confirmed by testing for the infections that cause these other conditions, and by a lack of response to antibiotic treatment. [2] Eosinophils tend to be found within the blisters and provide an important clue supporting bullous pemphigoid as the diagnosis.

Epidemiology and Demographics




Risk Factors

Natural History, Complications and Prognosis


Diagnostic Criteria

The gold standard for diagnosis is a punch biopsy from the area around the lesion that is examined by direct immunofluorescent staining, showing acantholytic cells. These can also be seen on a Tzanck smear. These cells are basically rounded, nucleated keratinocytes formed due to antibody mediated damage to cell adhesion protein: Desmoglein.

Physical Examination

On a physical exam, pemphigus vulgaris has flat bullae and a positive Nikolsky's sign.


Oral cavity

Laboratory Findings

Imaging Findings

Other Diagnostic Studies


Medical Therapy

Corticosteroids and other immunosuppressive drugs are the mainstay of treatment. Based on recent studies, corticosteroids can be used in Pulse Therapy/Supra-pharmacological doses once a month to decrease Hypothalamo-pituitary axis inhibition. IVIg, rituximab, mycophenolate mofetil, methotrexate, azathioprine, and cyclophosphamide have also been used with varying degrees of success. The development of new monoclonal antibodies holds promise for improved treatment of phemphigus in the future.



See also


  1. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  2. Kaplan DL (2009). "Dermclinic". Consultant. 49 (3).
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 3.9 "Dermatology Atlas".

Template:Vesiculobullous disease