Difference between revisions of "Bullous diseases"

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


DDx of bullous disease:<ref>TN07 D21-3.</ref>
=Overview=
*Bullous pemphigoid.
==DDx based on type==
===Subcorneal bullous disorders===
DDx '''with''' acantholysis:<ref name=pmid18418089>{{cite journal |author=Brinster NK |title=Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I) |journal=Adv Anat Pathol |volume=15 |issue=2 |pages=76–96 |year=2008 |month=March |pmid=18418089 |doi=10.1097/PAP.0b013e3181664e8d |url=}}</ref>
*[[Pemphigus foliaceous]].
*[[Bullous impetigo]].
*[[Staphylococcual scaled skin syndrome]].
 
DDx '''without''' acantholysis:DDx:<ref name=pmid18418089/>
*Subcorneal pustular demratosis (Sneddon-Wilkinson disease)
*Pustular psoriasis.
*Pustular drug eruption ([[acute generalized exanthematous pustulosis]]).
 
===Suprabasilar bullous disorders===
DDx:<ref name=pmid18418089/>
*[[Pemphigus vulgaris]].
*[[Pemphigus vulgaris]].
*[[Hailey-Hailey disease]] (benign familial pemphigus).
*[[Darier disease]].
*[[Grover disease]] (transient acantholytic dermatosis).
===Subepidermal bullous disorders===
DDx:<ref name=pmid18418089/>
*[[Bullous pemphigoid]].
*Cicatricial pemphigoid.
*[[Porphyria cutanea tarda]].
*[[Porphyria cutanea tarda]].
*[[Dermatitis herpetiformis]].
*Epidermolysis bullosa acquista.
*Epidermolysis bullosa.
*[[Dermatitis herpetiformsis]].
*Linear IgA disease.


Subcorneal bullous disease: (???)
Others:
*Pemphigus foliaceus.
*Insect bite.
*Pemphigus vegetans.
*Coma blister.
*[[Grover's disease]] (transient acantholytic dermatosis).
*Bullous [[systemic lupus erythematosus]].
*[[Darier's disease]].
*[[Hailey-Hailey disease]].


=Specific diseases=
==Bullous pemphigoid==
==Bullous pemphigoid==
===General===
===General===
Line 163: Line 184:
*Dyskeratosis.
*Dyskeratosis.


==See also==
=See also=
*[[Non-malignant skin disease]].
*[[Non-malignant skin disease]].
*[[Dermatopathology introduction]].
*[[Dermatopathology introduction]].
*[[Dermal cysts]].
*[[Dermal cysts]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Non-malignant skin disease]]
[[Category:Non-malignant skin disease]]
[[Category:Dermatopathology]]
[[Category:Dermatopathology]]

Revision as of 03:47, 20 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:

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:[2]

  • 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:[5]

  • 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.[7]

General

  • Genetic - autosomal dominant with incomplete penetration.[7]
    • Desmosomal defect - due to mutation in the gene ATP2C1.[7]

Clinical:

  • Chest.
  • Intertriginous regions. (???)
  • Typically presents individual in their 30s and 40s.[7]

Microscopic

Features:

  • Suprabasilar blistering.
  • Acanthosis (thick epidermis).

Notes:

  • Hair folicles spared.

DDx:

Dermatitis herpetiformis

General

Clinical:

  • Pruritis - intense.

Microscopic

Features:[8]

  • 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:[10]

  • 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:[11]

  • 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. ↑ 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. ↑ 7.0 7.1 7.2 7.3 URL: http://emedicine.medscape.com/article/1063224-overview. Accessed on: 9 September 2011.
  8. ↑ 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.
  9. ↑ URL: http://dermatology.cdlib.org/94/NYU/Nov2001/9.html. Accessed on: 21 March 2011.
  10. ↑ 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.
  11. ↑ S. Sade. 8 September 2011.
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