[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Skin](https://durgical.com/en/general-pathology/areas/skin/) › Pemphigus

# Pemphigus

- Lesion

In pemphigus, antibodies cut the glue between skin cells. Fragile blisters break easily and leave raw areas, often in the mouth.

Updated Nov 12, 2026

## Key points

- Mechanism: type II hypersensitivity; IgG4 and other IgG against desmoglein 3 (vulgaris) or desmoglein 1 (foliaceus).
- Pemphigus vulgaris: oral erosions first, flaccid blisters, Nikolsky sign; suprabasal split with basal cells left like a «row of tombstones».
- Direct immunofluorescence: intercellular («fishnet» or net-like) IgG and C3 throughout the epidermis.
- Pemphigus foliaceus: superficial subcorneal split, crusted erosions, no mucosa; endemic form (fogo selvagem) in rural Brazil and other parts of South America.
- Paraneoplastic pemphigus: severe stomatitis, lymphoma or Castleman disease; antibodies against plakins.
- Desmoglein compensation: mucosa relies on Dsg3, skin surface on Dsg1, which explains the distribution.

## What it is

Pemphigus is a group of autoimmune blistering diseases in which IgG autoantibodies bind desmogleins, the cadherins of desmosomes, causing acantholysis. Pemphigus vulgaris targets desmoglein 3 (with or without 1) and splits just above the basal layer, involving mucosa; pemphigus foliaceus targets desmoglein 1 and splits high, sparing mucosa. Paraneoplastic pemphigus accompanies lymphoid neoplasms. The level of the split explains how each blister looks.

## Structure

- Vulgaris: suprabasal acantholytic blister with rounded, detached keratinocytes in the cavity; basal cells remain attached to the basement membrane.
- Foliaceus: acantholysis at the granular layer, often with neutrophils; blister roof very thin.
- Perilesional skin is needed for direct immunofluorescence.

## Where the blister forms

| Level | Disease | Target | Immunofluorescence |
| --- | --- | --- | --- |
| [Subcorneal or granular](https://durgical.com/en/conditions/staphylococcal-scalded-skin-syndrome/) | Pemphigus foliaceus; staphylococcal scalded skin | Desmoglein 1 (antibody or toxin) | Intercellular IgG upper epidermis (foliaceus); none (toxin) |
| [Suprabasal](https://durgical.com/en/conditions/pemphigus-vulgaris/) | Pemphigus vulgaris | Desmoglein 3 (± 1) | Intercellular IgG, fishnet |
| [Subepidermal, lamina lucida](https://durgical.com/en/conditions/bullous-pemphigoid/) | Bullous pemphigoid | BP180, BP230 | Linear IgG and C3 at the basement membrane |
| [Subepidermal, dermal papillae](https://durgical.com/en/conditions/dermatitis-herpetiformis/) | Dermatitis herpetiformis | Epidermal transglutaminase | Granular IgA in papillary tips |
| [Below the lamina densa](https://durgical.com/en/conditions/epidermolysis-bullosa-acquisita/) | Epidermolysis bullosa acquisita; dystrophic EB | Type VII collagen | Linear IgG (acquired); none (inherited) |

## Clinical notes

- Before steroids, pemphigus vulgaris was often fatal through infection and fluid loss.
- Drug-induced pemphigus: penicillamine, captopril.
- Anti-CD20 therapy is now a first-line treatment.

## Memory aid

Pemphigus cuts the cell-to-cell glue: the basal row stays standing like tombstones while the rest of the wall floats away.

## Test yourself

Think of the answer, then tap the question to see it.

## Which antigen is targeted in pemphigus vulgaris?

Desmoglein 3 (often with desmoglein 1), a desmosomal cadherin.

## What is the direct immunofluorescence pattern in pemphigus?

Intercellular, net-like (fishnet) IgG and C3 deposition between keratinocytes.

## Why does pemphigus foliaceus spare the mouth?

It targets desmoglein 1, while oral mucosa relies mainly on desmoglein 3, which compensates.

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## Elsewhere in DurgiPedia

- [Type II hypersensitivity (antibody against cells)](https://durgical.com/en/immunology/type-ii-hypersensitivity/)
- [Immunofluorescence and the ANA test](https://durgical.com/en/immunology/immunofluorescence-and-the-ana-test/)
- [Where blisters form: pemphigus vs pemphigoid](https://durgical.com/en/histology/where-blisters-form-pemphigus-vs-pemphigoid/)
- [Cell junctions: tight, adherens, desmosomes, gap, hemidesmosomes](https://durgical.com/en/histology/cell-junctions-tight-adherens-desmosomes-gap-hemidesmosomes/)

## Connections

## Related 3

- [Bullous pemphigoid and dermatitis herpetiformis](https://durgical.com/en/general-pathology/bullous-pemphigoid-and-dermatitis-herpetiformis/)
- [Epidermolysis bullosa and porphyria](https://durgical.com/en/general-pathology/epidermolysis-bullosa-and-porphyria/)
- [Skin pathology: terms and reaction patterns](https://durgical.com/en/general-pathology/skin-pathology-terms-and-reaction-patterns/)

## Related conditions 4

- [Pemphigus vulgaris](https://durgical.com/en/conditions/pemphigus-vulgaris/)
- [Staphylococcal scalded skin syndrome](https://durgical.com/en/conditions/staphylococcal-scalded-skin-syndrome/)
- [Non-Hodgkin lymphoma](https://durgical.com/en/conditions/non-hodgkin-lymphoma/)
- [Bullous pemphigoid](https://durgical.com/en/conditions/bullous-pemphigoid/)

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: [Blisters](https://medlineplus.gov/blisters.html) (MedlinePlus, US National Library of Medicine, 2025).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/pemphigus/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/pemphigus/