Bullous diseases

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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]

Memory device - PhD + Grover = Pemphigus vulgaris, Hailey-Hailey, Darier, Grover.

Subepidermal bullous disorders

DDx:[1]

Others:

Mnemonic DELPHI:

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

Specific diseases

Pemphigus foliaceus

General

  • Autoimmune disease.[3]
    • Autoantibodies against desmoglein 1 only.

Note:

Microscopic

Features:

  • Subcorneal separation.
  • Acantholysis.
    • Separation of keratinocytes.

DDx:

Bullous pemphigoid

General

  • Less serious than pemphigus vulgaris.

Epidemiology:

  • Old people (60-80 year olds).

Clinical:

  • Extreme pruritis.

Etiology:

  • Antibodies to BPAG2 (a hemidesmosome protein).[5]

Notes:

Microscopic

Features:[6]

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

Notes:

Images:

DDx:

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.

Etiology:

  • Autoimmune disease.
    • Antibodies against: desmoglein 1 (DSG1)[9] and desmoglein 3 (DSG3).[4]

Microscopic

Features:[10]

  • Suprabasilar blistering.

DDx:

Images:

IF

  • Desmoglein 1 - abnormal.
  • Desmoglein 3 - abnormal.

Familial benign pemphigus

  • AKA Hailey-Hailey disease. Was described by two brothers - that's why it is Hailey-Hailey.[12]

General

  • Genetic - autosomal dominant with incomplete penetration.[12]
    • Desmosomal defect - due to mutation in the gene ATP2C1.[12]

Clinical:

  • Chest.
  • Intertriginous regions. (???)
  • Typically presents individual in their 30s and 40s.[12]

Microscopic

Features:

  • Suprabasilar blistering.
  • Acanthosis (thick epidermis).

Notes:

  • Hair folicles spared.

DDx:

Dermatitis herpetiformis

General

Clinical:

  • Pruritis - intense.

Microscopic

Features:[13]

  • 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:[15]

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

Images:

Epidermolysis bullosa acquisita

  • Abbreviated EBA

General

  • Autoimmune disease.
    • Antibodies to collagen type VII.[16]

Microscopic

Features:

  • Subepidermal bullae.

Epidermolysis bullosa

General

  • A group of inherited, bullous disorders.
  • Bullae form due to slight mechanical trauma.

Three major groupings:[17]

  1. Epidermolysis bullosa simplex (intraepidermal disease).
  2. Junctional epidermolysis bullosa (separation at DE junction; specifically central portion (lamina lucida)).
  3. Dystrophic epidermolysis bullosa (separation at DE junction; specifically deep to lamina densa).

Microscopic

Depends on subtype - either intraepidermal or subepidermal.

Grover disease

  • AKA transient acantholytic dermatosis.

General

  • Cause not known.
  • Associated with sun damaged skin, hospitalization and fever.[18]

Gross

  • Usually small ~ 50% less than 2 mm.[19]
  • Typically chest and back.[20]

Microscopic

Features:[21]

  • Subcorneal bullous disease.
  • Acanthosis.
  • Dyskeratosis.

DDx:

Images:

Acute generalized exanthematous pustulosis

  • Abbreviated AGEP.
  • AKA pustular drug eruption.

General

  • Drug reaction.

Clinical DDx:

Microscopic

Features:

  • Superficial dermis separates from underlying tissue. (???)

DDx:

Images:

Pemphigoid gestationis

  • AKA gestational pemphigoid.
  • Previously herpes gestationis.

General

  • Autoimmune condition in pregnancy.
    • Autoantibodies against XVII collagen.[22]
    • Fetus affected in ~5-10% of cases.[23]
  • Rare - est. 1/50,000 pregnancies.[24]

Treatment:

  • Corticosteroids.[25]

Microscopic

Features:

  • Subepidermal bullous disease.[26]
  • Eosinophils - abundant.

Images:

IF

  • C3 linear pattern at DE junction - diagnostic.[25]

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. ↑ James, KA.; Culton, DA.; Diaz, LA. (Jul 2011). "Diagnosis and clinical features of pemphigus foliaceus.". Dermatol Clin 29 (3): 405-12, viii. doi:10.1016/j.det.2011.03.012. PMID 21605805.
  4. ↑ 4.0 4.1 Online 'Mendelian Inheritance in Man' (OMIM) 169615
  5. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 607. ISBN 978-1416054542.
  6. ↑ 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.
  7. ↑ URL: http://dermatology.cdlib.org/94/NYU/Feb2002/8.html. Accessed on: 20 March 2011.
  8. ↑ URL: http://missinglink.ucsf.edu/lm/DermatologyGlossary/bullous_pemphigoid.html. Accessed on: 20 March 2011.
  9. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 125670
  10. ↑ 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.
  11. ↑ URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/pemphigus-vulgaris. Accessed on: 20 March 2011.
  12. ↑ 12.0 12.1 12.2 12.3 URL: http://emedicine.medscape.com/article/1063224-overview. Accessed on: 9 September 2011.
  13. ↑ 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.
  14. ↑ URL: http://dermatology.cdlib.org/94/NYU/Nov2001/9.html. Accessed on: 21 March 2011.
  15. ↑ 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.
  16. ↑ URL: http://emedicine.medscape.com/article/1063083-overview. Accessed on: 25 September 2011.
  17. ↑ URL: http://emedicine.medscape.com/article/1062939-overview. Accessed on: 25 September 2011.
  18. ↑ Quirk, CJ.; Heenan, PJ. (May 2004). "Grover's disease: 34 years on.". Australas J Dermatol 45 (2): 83-6; quiz 87-8. doi:10.1111/j.1440-0960.2004.054_1.x. PMID 15068451.
  19. ↑ Fernández-Figueras, MT.; Puig, L.; Cannata, P.; Cuatrecases, M.; Quer, A.; Ferrándiz, C.; Ariza, A. (Aug 2010). "Grover disease: a reappraisal of histopathological diagnostic criteria in 120 cases.". Am J Dermatopathol 32 (6): 541-9. doi:10.1097/DAD.0b013e3181c80cf9. PMID 20526170.
  20. ↑ {{Ref APBR|344 Q2
  21. ↑ S. Sade. 8 September 2011.
  22. ↑ Intong, LR.; Murrell, DF. (Jul 2011). "Pemphigoid gestationis: pathogenesis and clinical features.". Dermatol Clin 29 (3): 447-52, ix. doi:10.1016/j.det.2011.03.002. PMID 21605810.
  23. ↑ Tunzi, M.; Gray, GR. (Jan 2007). "Common skin conditions during pregnancy.". Am Fam Physician 75 (2): 211-8. PMID 17263216.
  24. ↑ Bedocs, PM.; Kumar, V.; Mahon, MJ. (Feb 2009). "Pemphigoid gestationis: a rare case and review.". Arch Gynecol Obstet 279 (2): 235-8. doi:10.1007/s00404-008-0687-3. PMID 18506459.
  25. ↑ 25.0 25.1 Campbell, SM.; Balazs, K.; Conroy, M. (Jul 2011). "Pemphigoid gestationis: a case report and review of the literature.". Cutis 88 (1): 21-6. PMID 21877502.
  26. ↑ Kolanko, E.; Bickle, K.; Keehn, C.; Glass, LF. (Mar 2004). "Subepidermal blistering disorders: a clinical and histopathologic review.". Semin Cutan Med Surg 23 (1): 10-8. PMID 15095911.
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