Pemphigus vulgaris: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{SI}}
{{CMG}}; {{AE}} {{KS}}
{{CMG}}; {{AE}} {{KS}}
{{SK}}
==Overview==
==Overview==


'''Pemphigus vulgaris''' is a chronic blistering skin disease with [[skin lesion]]s that are rarely [[pruritic]], but which are often painful.<ref name="Fitz2">Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.</ref>{{rp|561}}
'''Pemphigus vulgaris''' is a chronic blistering skin disease with [[skin lesion]]s that are rarely [[pruritic]], but which are often painful.<ref name="Fitz2">Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.</ref>{{rp|561}}
==Historical Perspective==
==Classification==


==Pathophysiology==
==Pathophysiology==
Line 10: Line 17:
[[Transudate|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 (skin)|epidermis]] and [[dermis]] (subepidermal bullae).
[[Transudate|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 (skin)|epidermis]] and [[dermis]] (subepidermal bullae).


==Diagnosis==
==Causes==
On a physical exam, pemphigus vulgaris has flat bullae and a positive [[Nikolsky's sign]]. The gold standard for diagnosis is a punch biopsy from the area around the lesion that is examined by direct [[immunofluorescence|immunofluorescent]] staining, showing [[acantholysis|acantholytic cell]]s. 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]].<br />


==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. <ref>{{cite journal |author= Kaplan DL |title= Dermclinic |journal=Consultant |volume=49 |issue=3 |year=2009 |url=http://www.consultantlive.com/display/article/10162/1387622?pageNumber=8}}</ref> [[Eosinophils]] tend to be found within the blisters and provide an important clue supporting bullous pemphigoid as the diagnosis.
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. <ref>{{cite journal |author= Kaplan DL |title= Dermclinic |journal=Consultant |volume=49 |issue=3 |year=2009 |url=http://www.consultantlive.com/display/article/10162/1387622?pageNumber=8}}</ref> [[Eosinophils]] tend to be found within the blisters and provide an important clue supporting bullous pemphigoid as the diagnosis.
==Epidemiology and Demographics==
===Age===
===Gender===
===Race===
==Risk Factors==
==Natural History, Complications and Prognosis==
==Diagnosis==
===Diagnostic Criteria===
The gold standard for diagnosis is a punch biopsy from the area around the lesion that is examined by direct [[immunofluorescence|immunofluorescent]] staining, showing [[acantholysis|acantholytic cell]]s. 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]].<br />


===Physical Examination===
===Physical Examination===
On a physical exam, pemphigus vulgaris has flat bullae and a positive [[Nikolsky's sign]].
====Skin====
====Skin====
=====Oral cavity=====
=====Oral cavity=====
Line 67: Line 88:


</gallery>
</gallery>
===Laboratory Findings===
===Imaging Findings===
===Other Diagnostic Studies===


==Treatment==
==Treatment==
===Medical Therapy===
[[Corticosteroid]]s 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.  
[[Corticosteroid]]s 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.  
===Surgery===
===Prevention===


== See also ==
== See also ==
Line 84: Line 116:
{{Vesiculobullous disease}}
{{Vesiculobullous disease}}


[[Category:Chronic blistering cutaneous conditions]]
[[Category:Needs content]]
[[Category:Rare diseases]]
[[Category:Rheumatology]]

Latest revision as of 18:38, 30 June 2016

WikiDoc Resources for Pemphigus vulgaris

Articles

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

Media

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 Trials.gov

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

NHS PRODIGY Guidance

FDA on Pemphigus vulgaris

CDC on Pemphigus vulgaris

Books

Books on Pemphigus vulgaris

News

Pemphigus vulgaris in the news

Be alerted to news on Pemphigus vulgaris

News trends on Pemphigus vulgaris

Commentary

Blogs on Pemphigus vulgaris

Definitions

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

International

Pemphigus vulgaris en Espanol

Pemphigus vulgaris en Francais

Business

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:

Overview

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

Historical Perspective

Classification

Pathophysiology

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).

Causes

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

Age

Gender

Race

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

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.

Skin

Oral cavity
Axilla

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

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.

Surgery

Prevention

See also

References

  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

Retrieved from "https://www.wikidoc.org/index.php?title=Pemphigus_vulgaris&oldid=1238751"