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pemphigus vulgaris

Monday 20 June 2005


- Pemphigus vulgaris with classic row of tombstone and DIF showing IgG chicken wire pattern of ICS deposits

- Deep follicular extension in pemphigus


Sera from patients with pemphigus contain antibodies (IgG) to intercellular cement substance of skin and mucous membranes. This phenomenon is the basis for direct and indirect diagnostic immunofluorescence testing of skin and serum, respectively.

Lesional sites show a characteristic netlike pattern of intercellular IgG deposits localized to sites of developed or incipient acantholysis. It is now known that the antibody in pemphigus vulgaris reacts with desmoglein-3, a component of the desmosomes that appear to bind keratinocytes together.

When the gene for desmoglein-3 is disrupted in genetically engineered mice, suprabasal blisters akin to pemphigus develop owing to lack of desmosome adhesion. This suggests a direct role for pemphigus autoantibodies in interfering with the function of this protein.

Some of the acantholytic process may also be the consequence of synthesis and liberation of a serine protease (plasminogen activator) by epidermal cells, an event that is triggered by the pemphigus antibody.

The relevant antibody in pemphigus foliaceus reacts with desmoglein-1, which is expressed in the uppermost epidermal layers, thus correlating with the characteristic subcorneal plane of blister formation in this variant.


- Payne AS, Hanakawa Y, Amagai M, Stanley JR. Desmosomes and disease: pemphigus and bullous impetigo. Curr Opin Cell Biol. 2004 Oct;16(5):536-43. PMID: 15363804

- Hashimoto T. Recent advances in the study of the pathophysiology of pemphigus. Arch Dermatol Res. 2003 Apr;295 Suppl 1:S2-11. PMID: 12677426

- Moll R, Moll I. Epidermal adhesion molecules and basement membrane components as target structures of autoimmunity. Virchows Arch. 1998 Jun;432(6):487-504. PMID: 9672190