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'''Bullous disease''' happens.  Dermatopathologists help diagnose it.
'''Bullous diseases''' are a subset of the large [[inflammatory skin diseases]] category.  Dermatopathologists help diagnose it.


DDx of bullous disease:<ref>TN07 D21-3.</ref>
An introduction to skin pathology is in the ''[[dermatopathology]]'' article.  An introduction to inflammatory skin lesions in the ''[[non-malignant skin disease]]'' article.
*Bullous pemphigoid.
 
*Pemphigus vulgaris.
Bullous disease of the [[lung]] is dealt with in ''[[lung bullae]]''.
*Porphyria cutanea tarda.
 
*Dermatitis herpetiformis.
=Overview=
*Epidermolysis bullosa.
==DDx based on type==
===Subcorneal bullous disorders===
DDx '''with''' acantholysis:<ref name=pmid18418089>{{cite journal |author=Brinster NK |title=Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I) |journal=Adv Anat Pathol |volume=15 |issue=2 |pages=76–96 |year=2008 |month=March |pmid=18418089 |doi=10.1097/PAP.0b013e3181664e8d |url=}}</ref>
*[[Pemphigus foliaceus]].
*[[Bullous impetigo]].
*[[Staphylococcal scalded skin syndrome]].
 
DDx '''without''' acantholysis:DDx:<ref name=pmid18418089/>
*Subcorneal pustular dermatosis (Sneddon-Wilkinson disease)
*Pustular psoriasis.
*Pustular drug eruption ([[acute generalized exanthematous pustulosis]]).
 
===Suprabasilar bullous disorders===
DDx:<ref name=pmid18418089/>
*[[Pemphigus vulgaris]].
*[[Hailey-Hailey disease]] (benign familial pemphigus).
*[[Darier disease]].
*[[Grover disease]] (transient acantholytic dermatosis).
 
Memory device - ''PhD'' + ''Grover'' = '''P'''emphigus vulgaris, '''H'''ailey-Hailey, '''D'''arier, '''G'''rover.
 
===Subepidermal bullous disorders===
DDx:<ref name=pmid18418089/>
*[[Bullous pemphigoid]].
*[[Cicatricial pemphigoid]].
*[[Porphyria cutanea tarda]].
*[[Epidermolysis bullosa acquista]].
*[[Dermatitis herpetiformis]].
*Linear IgA disease.
 
Others:
*Insect bite.
*Coma blister.
*Bullous [[systemic lupus erythematosus]].
 
Mnemonic ''DELPHI'':
*[[dermatitis herpetiformis|'''D'''ermatitis herpetiformis]].
*'''E'''pidermolysis bullosa acquisita.
*Bullous '''l'''upus erythematosis.
*'''P'''emphigoid, bullous.
*'''H'''erpes gestationis (now called ''[[pemphigoid gestationis]]'') - rare autoimmune bullous dermatosis of pregnancy, not related to HSV.<ref>URL: [http://emedicine.medscape.com/article/1063499-overview http://emedicine.medscape.com/article/1063499-overview]. Accessed on: 23 September 2011.</ref>
*Linear '''I'''gA disease.
 
=Specific diseases=
==Pemphigus foliaceus==
===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>
**Autoantibodies against ''desmoglein 1'' only.
 
Note:
*[[Pemphigus vulgaris]] has autoantibodies against ''desmoglein 1'' and ''desmoglein 3''.<ref name=omim169615>{{OMIM|169615}}</ref>
 
===Microscopic===
Features:
*Subcorneal separation.
*[[Acantholysis]].
**Separation of keratinocytes.
 
DDx:
*[[Staphylococcal scalded skin syndrome]].
*[[Bullous impetigo]].
 
===IF===
*Desmoglein 1 - abnormal.


==Bullous pemphigoid==
==Bullous pemphigoid==
===General===
===General===
*Less serious the ''pemphigus vulgaris''.
*Less serious than ''pemphigus vulgaris''.


Epidemiology:
Epidemiology:
*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:
*[[Pemphigus vulgaris]] = subepidermal.
*[[Pemphigus foliaceus]] = intraepidermal.


===Microscopic===
===Microscopic===
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*Epidermis not affect, i.e. non-acantholytic.
*Epidermis not affect, i.e. non-acantholytic.
*Linear Ig deposits along basement membrane.
*Linear Ig deposits along basement membrane.
*Early changes may be that of a ''[[dermal perivascular lymphoeosinophilic infiltration]]''.
Images:
*[http://dermatology.cdlib.org/94/NYU/Feb2002/021202-8b.jpg BP (dermatology.cdlib.org)].<ref>URL: [http://dermatology.cdlib.org/94/NYU/Feb2002/8.html http://dermatology.cdlib.org/94/NYU/Feb2002/8.html]. Accessed on: 20 March 2011.</ref>
*[http://missinglink.ucsf.edu/lm/DermatologyGlossary/img/Dermatology%20Glossary/Glossary%20Histo%20Images/bullous_pemphigoid_higher_power.jpg BP (ucsf.edu)].<ref>URL: [http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html]. Accessed on: 20 March 2011.</ref>
DDx:
*Bullous lupus.
*[[Dermal perivascular lymphoeosinophilic infiltration]].


==Pemphigus vulgaris==
==Pemphigus vulgaris==
*[[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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*Associated with [[thymoma]], myasthenia gravis, malignancy & D-penicillamine (used to Tx [[Wilson's disease]]).
*Associated with [[thymoma]], myasthenia gravis, malignancy & D-penicillamine (used to Tx [[Wilson's disease]]).
*Middle age.
*Middle age.
Etiology:
*Autoimmune disease.
**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)].
===IF===
*Desmoglein 1 - abnormal.
*Desmoglein 3 - abnormal.
==Familial benign pemphigus==
*AKA ''Hailey-Hailey disease''. Was described by two brothers - that's why it is ''Hailey-Hailey''.<ref name=emed_hailey>URL: [http://emedicine.medscape.com/article/1063224-overview http://emedicine.medscape.com/article/1063224-overview]. Accessed on: 9 September 2011.</ref>
===General===
*Genetic - autosomal dominant with incomplete penetration.<ref name=emed_hailey/>
**Desmosomal defect - due to mutation in the gene ''ATP2C1''.<ref name=emed_hailey/>
Clinical:
*Chest.
*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/>
===Microscopic===
Features:
*Suprabasilar blistering.
*[[Acanthosis]] (thick epidermis).


Notes:
Notes:
*Desmoglein 1, desmoglein 3 - abnormal.
*Hair folicles spared.
 
DDx:
*[[Pemphigus vulgaris]].


==Dermatitis herpetiformis==
==Dermatitis herpetiformis==
Assoc. with [[celiac sprue]].
===General===
*Associated with [[celiac sprue]].
 
Clinical:
*Pruritis - intense.
 
===Microscopic===
Features:<ref>{{Ref PBoD8|1196}}</ref>
*Subepidermal blistering.
*Clusters of neurophils (microabscesses) - at tips of dermal papillae - '''key feature'''.
*Basal cell injury (vacuolization).
 
Notes:
*Immunofluorescence - IgA deposits at dermal papillae.
 
Images:
*[http://dermatology.cdlib.org/94/NYU/Nov2001/112001-9b.jpg DH (dermatology.cdlib.org)].<ref>URL: [http://dermatology.cdlib.org/94/NYU/Nov2001/9.html http://dermatology.cdlib.org/94/NYU/Nov2001/9.html]. Accessed on: 21 March 2011.</ref>
*[http://www.dermpedia.org/files/images/Image49_HE.jpg DH (dermpedia.org)].


==Porphyria cutanea tarda==
==Porphyria cutanea tarda==
===Etiology===
===General===
Etiology:
*Genetic, autosomal dominant.
*Genetic, autosomal dominant.


===Appearance===
Treatment:
*Vesicles/bullae in photoexposed areas subjected to trauma.
*D/C aggravating substances (see below) - phlebotomy, hydroxychloroquine if phlebotomy contraindicated.


===Associations===
Note:
Medications/Substances:
*Fits into a larger category of ''porphyria''.
====Associations====
Medications/substances:
*[[EtOH]], Rx (estrogen, [[NSAID]]s).
*[[EtOH]], Rx (estrogen, [[NSAID]]s).


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*[[HIV]], [[hepatitis C]].  
*[[HIV]], [[hepatitis C]].  


===Treatment===  
===Gross===
*d/c aggravating substances (listed above), phlebotomy, hydroxychloroquine if phlebotomy contraindicated.
*In photoexposed areas subjected to trauma.
 
===Microscopic===
Features:<ref>{{Ref PBoD8|1197}}</ref>
*Subepidermal vesicles.
*Thickening of superficial dermal blood vessels.
 
Images:
*[http://www.dermpedia.org/files/images/Image1_4.jpg Subepidermal blistering with thick vessels (dermpedia.org)].
 
==Epidermolysis bullosa acquisita==
*Abbreviated ''EBA''
 
===General===
*Autoimmune disease.
**Antibodies to collagen type VII.<ref>URL: [http://emedicine.medscape.com/article/1063083-overview http://emedicine.medscape.com/article/1063083-overview]. Accessed on: 25 September 2011.</ref>
 
===Microscopic===
Features:
*Subepidermal bullae.


==Epidermolysis bullosa==
==Epidermolysis bullosa==
*Inherited, bullae & erosions from slight mechanical trauma.
===General===
*A group of inherited, bullous disorders.
*Bullae form due to slight mechanical trauma.
 
Three major groupings:<ref>URL: [http://emedicine.medscape.com/article/1062939-overview http://emedicine.medscape.com/article/1062939-overview]. Accessed on: 25 September 2011.</ref>
#Epidermolysis bullosa simplex (intraepidermal disease).
#Junctional epidermolysis bullosa (separation at DE junction; specifically central portion (lamina lucida)).
#Dystrophic epidermolysis bullosa (separation at DE junction; specifically deep to lamina densa).
 
===Microscopic===
Depends on subtype - either intraepidermal ''or'' subepidermal.
 
==Grover disease==
*[[AKA]] ''transient acantholytic dermatosis''.
 
===General===
*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===
*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===
Features:<ref>S. Sade. 8 September 2011.</ref>
*Subcorneal bullous disease.
*Acanthosis.
*Dyskeratosis.
 
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>
#[[Pemphigus vulgaris]].
#[[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==
*Abbreviated ''AGEP''.
*[[AKA]] pustular drug eruption.
 
===General===
*Drug reaction.
 
Clinical DDx:
*[[Erythema multiforme]].
*[[Stevens-Johnson syndrome]] (SJS) / [[toxic epidermal necrolysis]] (TEN).
 
===Microscopic===
Features:
*Superficial dermis separates from underlying tissue. (???)
 
DDx:
*[[Staphylococcal scalded skin syndrome]].
 
Images:
*[http://www.dermpedia.org/case/acute-generalized-exanthematous-pustulosis AGEP (dermpedia.org)].
 
==Pemphigoid gestationis==
*[[AKA]] ''gestational pemphigoid''.
*Previously ''herpes gestationis''.
 
===General===
*Autoimmune condition in pregnancy.
**Autoantibodies against XVII collagen.<ref name=pmid21605810>{{Cite journal  | last1 = Intong | first1 = LR. | last2 = Murrell | first2 = DF. | title = Pemphigoid gestationis: pathogenesis and clinical features. | journal = Dermatol Clin | volume = 29 | issue = 3 | pages = 447-52, ix | month = Jul | year = 2011 | doi = 10.1016/j.det.2011.03.002 | PMID = 21605810 }}</ref>
**Fetus affected in ~5-10% of cases.<ref name=pmid17263216>{{Cite journal  | last1 = Tunzi | first1 = M. | last2 = Gray | first2 = GR. | title = Common skin conditions during pregnancy. | journal = Am Fam Physician | volume = 75 | issue = 2 | pages = 211-8 | month = Jan | year = 2007 | doi =  | PMID = 17263216 | URL = http://www.aafp.org/afp/2007/0115/p211.html }}</ref>
*Rare - est. 1/50,000 pregnancies.<ref name=pmid18506459>{{Cite journal  | last1 = Bedocs | first1 = PM. | last2 = Kumar | first2 = V. | last3 = Mahon | first3 = MJ. | title = Pemphigoid gestationis: a rare case and review. | journal = Arch Gynecol Obstet | volume = 279 | issue = 2 | pages = 235-8 | month = Feb | year = 2009 | doi = 10.1007/s00404-008-0687-3 | PMID = 18506459 }}</ref>
 
Treatment:
*Corticosteroids.<ref name=pmid21877502/>
===Microscopic===
Features:
*Subepidermal bullous disease.<ref>{{Cite journal  | last1 = Kolanko | first1 = E. | last2 = Bickle | first2 = K. | last3 = Keehn | first3 = C. | last4 = Glass | first4 = LF. | title = Subepidermal blistering disorders: a clinical and histopathologic review. | journal = Semin Cutan Med Surg | volume = 23 | issue = 1 | pages = 10-8 | month = Mar | year = 2004 | doi =  | PMID = 15095911 }}</ref>
*Eosinophils - abundant.
 
====Images====
<gallery>
Image:Pemphigoid_gestationis_-_low_mag.jpg | PG - low 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===
*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=
*[[Non-malignant skin disease]].
*[[Non-malignant skin disease]].
*[[Dermatopathology introduction]].
*[[Dermatopathology introduction]].
*[[Dermal cysts]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Non-malignant skin disease]]
[[Category:Non-malignant skin disease]]
[[Category:Dermatopathology]]
[[Category:Dermatopathology]]
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