Bullous diseases
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.
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
Etiology
- Genetic, autosomal dominant.
Appearance
- Vesicles/bullae in photoexposed areas subjected to trauma.
Associations
Medications/Substances:
Non-infection chronic conditions:
- DM.
Infections:
Treatment
- d/c aggravating substances (listed above), phlebotomy, hydroxychloroquine if phlebotomy contraindicated.
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.