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Pathology · patient case question

Pemphigus vulgaris affects what?

Patient case: Pemphigus vulgaris and mucous membrane pemphigoid

Scenario: Questions on the vesiculobullous diseases.

  1. Hemidesmosomes
  2. Desmosomes
  3. Type VII collagen anchoring fibrils
  4. The basement membrane lamina densa
Show answer and explanation

Correct answer: B. Desmosomes

  • A. Hemidesmosomes anchor the BASAL cells to the basement membrane and are the target in MUCOUS MEMBRANE PEMPHIGOID (antibodies to BP180/BP230 and laminin 332), which gives a SUBEPITHELIAL split.
  • B. Correct. Pemphigus vulgaris is caused by IgG autoantibodies against DESMOGLEIN 3 (and desmoglein 1), the cadherin proteins of the DESMOSOMES that bind adjacent keratinocytes together. Loss of these connections produces ACANTHOLYSIS and an INTRAEPITHELIAL (suprabasilar) split, leaving the classic 'row of tombstones' basal layer and free-floating Tzanck cells.
  • C. Type VII collagen anchoring fibrils are targeted in epidermolysis bullosa acquisita, a different and much rarer condition.
  • D. The lamina densa is a basement membrane layer; it is not the primary target in pemphigus.

Note: Memory hook: PemphiGUS = GLUE between cells (desmosomes, intraepithelial split, more serious/potentially fatal). PemphiGOID = GROUND/floor beneath the cells (hemidesmosomes, subepithelial split).

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