Bullous diseases

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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

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

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:

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

  1. TN07 D21-3.
  2. 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.
  3. URL: http://dermatology.cdlib.org/94/NYU/Feb2002/8.html. Accessed on: 20 March 2011.
  4. URL: http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html. Accessed on: 20 March 2011.
  5. 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.
  6. URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.
  7. 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.
  8. URL: http://dermatology.cdlib.org/94/NYU/Nov2001/9.html. Accessed on: 21 March 2011.