Difference between revisions of "Bullous diseases"

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An introduction to skin pathology is in the ''[[dermatopathology]]'' article.  An introduction to inflammatory skin lesions in the ''[[non-malignant skin disease]]'' article.
An introduction to skin pathology is in the ''[[dermatopathology]]'' article.  An introduction to inflammatory skin lesions in the ''[[non-malignant skin disease]]'' article.
Bullous disease of the [[lung]] is dealt with in ''[[lung bullae]]''.


=Overview=
=Overview=
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===General===
===General===
*Autoimmune disease.<ref name=pmid21605805>{{Cite journal  | last1 = James | first1 = KA. | last2 = Culton | first2 = DA. | last3 = Diaz | first3 = LA. | title = Diagnosis and clinical features of pemphigus foliaceus. | journal = Dermatol Clin | volume = 29 | issue = 3 | pages = 405-12, viii | month = Jul | year = 2011 | doi = 10.1016/j.det.2011.03.012 | PMID = 21605805 }}</ref>
*Autoimmune disease.<ref name=pmid21605805>{{Cite journal  | last1 = James | first1 = KA. | last2 = Culton | first2 = DA. | last3 = Diaz | first3 = LA. | title = Diagnosis and clinical features of pemphigus foliaceus. | journal = Dermatol Clin | volume = 29 | issue = 3 | pages = 405-12, viii | month = Jul | year = 2011 | doi = 10.1016/j.det.2011.03.012 | PMID = 21605805 }}</ref>
**Autoantibodies against desmoglein-1.
**Autoantibodies against ''desmoglein 1'' only.
 
Note:
*[[Pemphigus vulgaris]] has autoantibodies against ''desmoglein 1'' and ''desmoglein 3''.<ref name=omim169615>{{OMIM|169615}}</ref>


===Microscopic===
===Microscopic===
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*[[Staphylococcal scalded skin syndrome]].
*[[Staphylococcal scalded skin syndrome]].
*[[Bullous impetigo]].
*[[Bullous impetigo]].
===IF===
*Desmoglein 1 - abnormal.


==Bullous pemphigoid==
==Bullous pemphigoid==
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*Old people (60-80 year olds).
*Old people (60-80 year olds).


Clinical
Clinical:
*Extreme pruritis.
*Extreme pruritis.


Etiology:
Etiology:
*Antibodies to BPAG2.
*Antibodies to BPAG2 (a hemidesmosome protein).<ref name=Ref_PCPBoD8_607>{{Ref PCPBoD8|607}}</ref>


Notes:
Notes:
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*[[AKA]] ''pemphigus''.
*[[AKA]] ''pemphigus''.
===General===
===General===
*May lead to blindness.
*Oral lesion is classically: ''first to show & last to go''.
**Oral lesions usually precede the skin lesions.
Classic presentation:
Classic presentation:
*Mouth lesions.
*Mouth lesions.
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Etiology:
Etiology:
*Autoimmune disease.  
*Autoimmune disease.  
**Antibodies against: desmoglein 1, desmoglein 3.
**Antibodies against: ''desmoglein 1'' (DSG1)<ref name=omim125670>{{OMIM|125670}}</ref> and ''desmoglein 3'' (DSG3).<ref name=omim169615>{{OMIM|169615}}</ref>


===Microscopic===
===Microscopic===
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*Suprabasilar blistering.
*Suprabasilar blistering.


DDx: [[Hailey-Hailey disease]].
DDx:  
*[[Hailey-Hailey disease]].
*[[Darier disease]].
*[[Grover disease]].


Images:
Images:
*[http://www.dermpedia.org/files/images/pemphigus_vulgaris_3.jpg PV (dermpedia.org)].<ref>URL: [http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris]. Accessed on: 20 March 2011.</ref>
*[http://www.dermpedia.org/files/images/pemphigus_vulgaris_3.jpg PV (dermpedia.org)].<ref>URL: [http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris]. Accessed on: 20 March 2011.</ref>
*[http://commons.wikimedia.org/wiki/File:Pemphigus_vulgaris_-_intermed_mag.jpg Pemphigus vulgaris - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Pemphigus_vulgaris_-_high_mag.jpg Pemphigus vulgaris - high mag. (WC)].


Notes:
===IF===
*Desmoglein 1, desmoglein 3 - abnormal.
*Desmoglein 1 - abnormal.
*Desmoglein 3 - abnormal.


==Familial benign pemphigus==
==Familial benign pemphigus==
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Clinical:
Clinical:
*Chest.
*Chest.
*Intertriginous regions. (???)
*Intertriginous regions (only) - where skin rubs together, e.g. skin folds of the breast, axilla.
*Typically presents individual in their 30s and 40s.<ref name=emed_hailey/>
*Typically presents individual in their 30s and 40s.<ref name=emed_hailey/>


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Features:  
Features:  
*Suprabasilar blistering.
*Suprabasilar blistering.
*Acanthosis (thick epidermis).
*[[Acanthosis]] (thick epidermis).


Notes:
Notes:
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==Grover disease==
==Grover disease==
*[[AKA]] transient acantholytic dermatosis.
*[[AKA]] ''transient acantholytic dermatosis''.


===General===
===General===
*Genetic. (???)
*Cause not known.
*Associated with sun damaged skin, hospitalization and fever.<ref name=pmid15068451>{{Cite journal  | last1 = Quirk | first1 = CJ. | last2 = Heenan | first2 = PJ. | title = Grover's disease: 34 years on. | journal = Australas J Dermatol | volume = 45 | issue = 2 | pages = 83-6; quiz 87-8 | month = May | year = 2004 | doi = 10.1111/j.1440-0960.2004.054_1.x | PMID = 15068451 }}</ref>
 
Clinical:<ref name=pmid16939188>{{Cite journal  | last1 = Hanson | first1 = M. | last2 = Hsu | first2 = S. | title = Pruritic papules on the chest and back. Grover's disease. | journal = Am Fam Physician | volume = 74 | issue = 4 | pages = 641-2 | month = Aug | year = 2006 | doi =  | PMID = 16939188 | url = http://www.aafp.org/afp/2006/0815/p641.html }}</ref>
*Pruritis (itchy).
*Usually self-limited.
*Treatment for symptoms.


===Gross===
===Gross===
*Usually small ~ 50% less than 2 mm.<ref name=pmid20526170>{{Cite journal  | last1 = Fernández-Figueras | first1 = MT. | last2 = Puig | first2 = L. | last3 = Cannata | first3 = P. | last4 = Cuatrecases | first4 = M. | last5 = Quer | first5 = A. | last6 = Ferrándiz | first6 = C. | last7 = Ariza | first7 = A. | title = Grover disease: a reappraisal of histopathological diagnostic criteria in 120 cases. | journal = Am J Dermatopathol | volume = 32 | issue = 6 | pages = 541-9 | month = Aug | year = 2010 | doi = 10.1097/DAD.0b013e3181c80cf9 | PMID = 20526170 }}</ref>
*Usually small ~ 50% less than 2 mm.<ref name=pmid20526170>{{Cite journal  | last1 = Fernández-Figueras | first1 = MT. | last2 = Puig | first2 = L. | last3 = Cannata | first3 = P. | last4 = Cuatrecases | first4 = M. | last5 = Quer | first5 = A. | last6 = Ferrándiz | first6 = C. | last7 = Ariza | first7 = A. | title = Grover disease: a reappraisal of histopathological diagnostic criteria in 120 cases. | journal = Am J Dermatopathol | volume = 32 | issue = 6 | pages = 541-9 | month = Aug | year = 2010 | doi = 10.1097/DAD.0b013e3181c80cf9 | PMID = 20526170 }}</ref>
*Typically chest and back.<ref>{{Ref APBR|344 Q2</ref>


===Microscopic===
===Microscopic===
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*Dyskeratosis.
*Dyskeratosis.


Images:
DDx:<ref name=pmid10089990>{{Cite journal  | last1 = Davis | first1 = MD. | last2 = Dinneen | first2 = AM. | last3 = Landa | first3 = N. | last4 = Gibson | first4 = LE. | title = Grover's disease: clinicopathologic review of 72 cases. | journal = Mayo Clin Proc | volume = 74 | issue = 3 | pages = 229-34 | month = Mar | year = 1999 | doi = 10.4065/74.3.229 | PMID = 10089990 }}</ref>
*[http://commons.wikimedia.org/wiki/File:Transient_acantholytic_dermatosis_-_high_mag.jpg Grover disease - high mag. (WC)].
#[[Pemphigus vulgaris]].
*[http://commons.wikimedia.org/wiki/File:Transient_acantholytic_dermatosis_-_very_high_mag.jpg Grover disease - very high mag. (WC)].
#[[Darier disease]].
 
====Images====
<gallery>
Image:Transient acantholytic dermatosis - intermed mag.jpg | Grover disease - intermed. mag. (WC)
Image:Transient_acantholytic_dermatosis_-_high_mag.jpg | Grover disease - high mag. (WC)
Image:Transient_acantholytic_dermatosis_-_very_high_mag.jpg | Grover disease - very high mag. (WC)
</gallery>


==Acute generalized exanthematous pustulosis==
==Acute generalized exanthematous pustulosis==
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*Eosinophils - abundant.
*Eosinophils - abundant.


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Pemphigoid_gestationis_-_low_mag.jpg PG - low mag. (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Pemphigoid_gestationis_-_high_mag.jpg PG - high mag. (WC)].
Image:Pemphigoid_gestationis_-_low_mag.jpg | PG - low mag. (WC)
*[http://commons.wikimedia.org/wiki/File:Pemphigoid_gestationis_-_very_high_mag.jpg PG - very high mag. (WC)].
Image:Pemphigoid_gestationis_-_high_mag.jpg | PG - high mag. (WC)
Image:Pemphigoid_gestationis_-_very_high_mag.jpg | PG - very high mag. (WC)
</gallery>


===IF===
===IF===
*C3 linear pattern at DE junction - diagnostic.<ref name=pmid21877502>{{Cite journal  | last1 = Campbell | first1 = SM. | last2 = Balazs | first2 = K. | last3 = Conroy | first3 = M. | title = Pemphigoid gestationis: a case report and review of the literature. | journal = Cutis | volume = 88 | issue = 1 | pages = 21-6 | month = Jul | year = 2011 | doi =  | PMID = 21877502 }}</ref>
*C3 linear pattern at DE junction - diagnostic.<ref name=pmid21877502>{{Cite journal  | last1 = Campbell | first1 = SM. | last2 = Balazs | first2 = K. | last3 = Conroy | first3 = M. | title = Pemphigoid gestationis: a case report and review of the literature. | journal = Cutis | volume = 88 | issue = 1 | pages = 21-6 | month = Jul | year = 2011 | doi =  | PMID = 21877502 }}</ref>
==Bullous Arthropod Assault==
[[File:30MR17 dp sl 1.png|Bullous arthropod assault]]
[[File:30MR17 dp sl 2.png|Bullous arthropod assault]]
[[File:30MR17 dp sl 3.png|Bullous arthropod assault]]<br>
Bullous arthropod assault. A. Beneath intraepidermal blistering lies superficial and deep inflammation with extension into fat. B. The vesicle shows spongiotic epidermis at base and multiple eosinophils. C. Eosinophils are prominent in dermal and adipose tissue inflammation.


=See also=
=See also=
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