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

Epidemiology

Old people (60-80 year olds).

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:^[2]

Suprabasilar blistering.

DDx: Hailey-Hailey disease.

Images:

PV (dermpedia.org).^[3]

Notes:

Desmoglein 1, desmoglein 3 - abnormal.

Dermatitis herpetiformis

Assoc. with celiac sprue.

Porphyria cutanea tarda

Etiology

Genetic, autosomal dominant.

Appearance

Vesicles/bullae in photoexposed areas subjected to trauma.

Associations

Medications/Substances:

EtOH, Rx (estrogen, NSAIDs).

Non-infection chronic conditions:

DM.

Infections:

HIV, hepatitis C.

Treatment

d/c aggravating substances (listed above), phlebotomy, hydroxychloroquine if phlebotomy contraindicated.

Epidermolysis bullosa

Inherited, bullae & erosions from slight mechanical trauma.

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. 1193. ISBN 978-1416031215.
↑ URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.

[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. 1193. ISBN 978-1416031215.

[3] URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.

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