Difference between revisions of "Bullous diseases"

From Libre Pathology
Jump to navigation Jump to search
Line 36: Line 36:
*Coma blister.
*Coma blister.
*Bullous [[systemic lupus erythematosus]].
*Bullous [[systemic lupus erythematosus]].
Mnemonic ''DELPHI'':
*'''D'''ermatitis herpetiformis.
*'''E'''pidermolysis bullosa acquisita.
*Bullous '''l'''upus erythematosis.
*'''P'''emphigoid, bullous.
*'''H'''erpes 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=
=Specific diseases=

Revision as of 22:48, 25 September 2011

Bullous diseases are a subset of the large inflammatory skin diseases category. Dermatopathologists help diagnose it.

An introduction to skin pathology is in the dermatopathology article. An introduction to inflammatory skin lesions in the non-malignant skin disease article.

Overview

DDx based on type

Subcorneal bullous disorders

DDx with acantholysis:[1]

DDx without acantholysis:DDx:[1]

Suprabasilar bullous disorders

DDx:[1]

Subepidermal bullous disorders

DDx:[1]

Others:

Mnemonic DELPHI:

  • Dermatitis herpetiformis.
  • Epidermolysis bullosa acquisita.
  • Bullous lupus erythematosis.
  • Pemphigoid, bullous.
  • Herpes gestationis - rare autoimmune bullous dermatosis of pregnancy, not related to HSV.[2]
  • Linear IgA disease.

Specific diseases

Bullous pemphigoid

General

  • Less serious than pemphigus vulgaris.

Epidemiology:

  • Old people (60-80 year olds).

Clinical

  • Extreme pruritis.

Etiology:

  • Antibodies to BPAG2.

Microscopic

Features:[3]

  • Subepidermal blisters.
  • +/-Lymphocytes.
  • +/-Eosinophils.
  • +/-Neutrophils.

Notes:

  • Epidermis not affect, i.e. non-acantholytic.
  • Linear Ig deposits along basement membrane.

Images:

DDx:

  • Bullous lupus.

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:[6]

  • Suprabasilar blistering.

DDx: Hailey-Hailey disease.

Images:

Notes:

  • Desmoglein 1, desmoglein 3 - abnormal.

Familial benign pemphigus

  • AKA Hailey-Hailey disease. Was described by two brothers - that's why it is Hailey-Hailey.[8]

General

  • Genetic - autosomal dominant with incomplete penetration.[8]
    • Desmosomal defect - due to mutation in the gene ATP2C1.[8]

Clinical:

  • Chest.
  • Intertriginous regions. (???)
  • Typically presents individual in their 30s and 40s.[8]

Microscopic

Features:

  • Suprabasilar blistering.
  • Acanthosis (thick epidermis).

Notes:

  • Hair folicles spared.

DDx:

Dermatitis herpetiformis

General

Clinical:

  • Pruritis - intense.

Microscopic

Features:[9]

  • 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:

Infections:

Gross

  • In photoexposed areas subjected to trauma.

Microscopic

Features:[11]

  • Subepidermal vesicles.
  • Thickening of superficial dermal blood vessels.

Images:

Epidermolysis bullosa

  • Inherited, bullae & erosions from slight mechanical trauma.

Grover disease

  • AKA transient acantholytic dermatosis.

General

  • Genetic. (???)

Microscopic

Features:[12]

  • Subcorneal bullous disease.
  • Acanthosis.
  • Dyskeratosis.

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 Brinster NK (March 2008). "Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I)". Adv Anat Pathol 15 (2): 76–96. doi:10.1097/PAP.0b013e3181664e8d. PMID 18418089.
  2. ↑ URL: http://emedicine.medscape.com/article/1063499-overview. Accessed on: 23 September 2011.
  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.
  4. ↑ URL: http://dermatology.cdlib.org/94/NYU/Feb2002/8.html. Accessed on: 20 March 2011.
  5. ↑ URL: http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html. Accessed on: 20 March 2011.
  6. ↑ 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.
  7. ↑ URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.
  8. ↑ 8.0 8.1 8.2 8.3 URL: http://emedicine.medscape.com/article/1063224-overview. Accessed on: 9 September 2011.
  9. ↑ 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.
  10. ↑ URL: http://dermatology.cdlib.org/94/NYU/Nov2001/9.html. Accessed on: 21 March 2011.
  11. ↑ 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.
  12. ↑ S. Sade. 8 September 2011.
Retrieved from "https://librepathology.org/w/index.php?title=Bullous_diseases&oldid=7832"