Pemphigus foliaceus

Last Updated: 2022-03-11

Author(s): Anzengruber F., Navarini A.

ICD11: EB40.1

Anti-DSG1 (&3) autoimmune disease

Most common in middle age, but it has also been described from 18 months to 70 years of age.

  • Classical variant
  • Pemphigus erythematosus Senear Usher

Autoimmune reaction with antibodies against DSG1 (&3)

Frequently not perceptible as bullae, erythematous, partly erosive, finely scaling areas focussed on the seborrhoeic zones. May look similar to eczema. May itch and burn. Typical onset is after emotional stress (33% of cases), otherwise risk factors sunlight, heat.

Biopsy with direct immunofluorescence

Anti-desmoglein 1 & 3

Seborrhoeic zones, but can affect the whole body. Does not go on the mucous membranes.

Burning and itching blisters developed, mechanical? Trigger stress? Muscle weakness?

Superficial intraepidermal bullae. There is nothing in the cavities, or fibrin, or even neutrophils. Dyskeratotic cells in the str. granulosum are suggestive of pemphigus foliaceus. Early on, eosinophilic spongiosis may also occur, in which case attention should be paid to the differential diagnosis of bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis.

Direct immunofluorescence positive in almost 100%, intercellular immunoglobulins and complement in the epidermis.


None possible.

Relatively good, can give punctual recurrences.

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