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Latest revision as of 18:38, 30 June 2016

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

Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]

Axilla

Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]
Pemphigus vulgaris. With permission from Dermatology Atlas.^[3]

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

References

↑ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
↑ Kaplan DL (2009). "Dermclinic". Consultant. 49 (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

[Fitz2-1] 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).

[Dermatology_Atlas-3] 3.0 ^3.1 ^3.2 ^3.3 ^3.4 ^3.5 ^3.6 ^3.7 ^3.8 ^3.9 "Dermatology Atlas".

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
 __NOTOC__
+{{SI}}
 {{CMG}}; {{AE}} {{KS}}
+{{SK}}
 ==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}}
+==Historical Perspective==
+==Classification==
 ==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).
-==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.
+==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===
+On a physical exam, pemphigus vulgaris has flat bullae and a positive [[Nikolsky's sign]].
 ====Skin====
 =====Oral cavity=====
 <gallery>
-Image:Pemphigus vulgaris01.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris01.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris02.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris02.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris03.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris03.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris04.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris04.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris05.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris05.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
@@ Line 39: / Line 60: @@
 <gallery>
-Image:Pemphigus vulgaris06.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris06.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris07.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris07.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris08.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris08.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris09.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris09.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
-Image:Pemphigus vulgaris10.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Pemphigus vulgaris10.jpg|Pemphigus vulgaris. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
@@ Line 67: / Line 88: @@
 </gallery>
+===Laboratory Findings===
+===Imaging Findings===
+===Other Diagnostic Studies===
 ==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.
+===Surgery===
+===Prevention===
 == See also ==
@@ Line 84: / Line 116: @@
 {{Vesiculobullous disease}}
-[[Category:Chronic blistering cutaneous conditions]]
+[[Category:Needs content]]
-[[Category:Rare diseases]]
+[[Category:Rheumatology]]