Difference between revisions of "Bullous diseases"
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==Porphyria cutanea tarda== | ==Porphyria cutanea tarda== | ||
=== | ===General=== | ||
Etiology: | |||
*Genetic, autosomal dominant. | *Genetic, autosomal dominant. | ||
Treatment: | |||
* | *D/C aggravating substances (see below) - phlebotomy, hydroxychloroquine if phlebotomy contraindicated. | ||
===Associations=== | Note: | ||
Medications/ | *Fits into a larger category of ''porphyria''. | ||
====Associations==== | |||
Medications/substances: | |||
*[[EtOH]], Rx (estrogen, [[NSAID]]s). | *[[EtOH]], Rx (estrogen, [[NSAID]]s). | ||
| Line 99: | Line 102: | ||
*[[HIV]], [[hepatitis C]]. | *[[HIV]], [[hepatitis C]]. | ||
=== | ===Gross=== | ||
* | *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== | ==Epidermolysis bullosa== | ||
Revision as of 04:20, 21 March 2011
Bullous disease happens. Dermatopathologists help diagnose it.
DDx of bullous disease:[1]
- Bullous pemphigoid.
- Pemphigus vulgaris.
- Porphyria cutanea tarda.
- Dermatitis herpetiformis.
- Epidermolysis bullosa.
Bullous pemphigoid
General
- Less serious than pemphigus vulgaris.
Epidemiology:
- Old people (60-80 year olds).
Clinical
- Extreme pruritis.
Etiology:
- Antibodies to BPAG2.
Microscopic
Features:[2]
- Subepidermal blisters.
- +/-Lymphocytes.
- +/-Eosinophils.
- +/-Neutrophils.
Notes:
- Epidermis not affect, i.e. non-acantholytic.
- Linear Ig deposits along basement membrane.
Images:
Pemphigus vulgaris
- AKA pemphigus.
General
Classic presentation:
- Mouth lesions.
- Non-pruritic.
Treatment:
- Prednisone then steroid sparing agent.
Epidemiology:
- Associated with thymoma, myasthenia gravis, malignancy & D-penicillamine (used to Tx Wilson's disease).
- Middle age.
Microscopic
Features:[5]
- Suprabasilar blistering.
DDx: Hailey-Hailey disease.
Images:
Notes:
- Desmoglein 1, desmoglein 3 - abnormal.
Dermatitis herpetiformis
General
- Associated with celiac sprue.
Clinical:
- Pruritis - intense.
Microscopic
Features:[7]
- 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:
Porphyria cutanea tarda
General
Etiology:
- Genetic, autosomal dominant.
Treatment:
- D/C aggravating substances (see below) - phlebotomy, hydroxychloroquine if phlebotomy contraindicated.
Note:
- Fits into a larger category of porphyria.
Associations
Medications/substances:
Non-infection chronic conditions:
- DM.
Infections:
Gross
- In photoexposed areas subjected to trauma.
Microscopic
Features:[9]
- Subepidermal vesicles.
- Thickening of superficial dermal blood vessels.
Images:
Epidermolysis bullosa
- Inherited, bullae & erosions from slight mechanical trauma.
See also
References
- ↑ TN07 D21-3.
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1195. ISBN 978-1416031215.
- ↑ URL: http://dermatology.cdlib.org/94/NYU/Feb2002/8.html. Accessed on: 20 March 2011.
- ↑ URL: http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html. Accessed on: 20 March 2011.
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1193. ISBN 978-1416031215.
- ↑ URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1196. ISBN 978-1416031215.
- ↑ URL: http://dermatology.cdlib.org/94/NYU/Nov2001/9.html. Accessed on: 21 March 2011.
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1197. ISBN 978-1416031215.