Pemphigus



Pemphigus is a life-threatening recurrent autoimmune disease with intraepidermal blisters production on the skin and on the mucous membranes, which mostly affects older people, between 30 and 60 years.

Pemphigus vulgaris
An acquired, chronic autoimmune disease. In primary blisters formation on mucous membranes it is possible to detect IgG against keratinocyte membrane antigens, desmoglein 3, in primary skin form - against desmoglein 1. Direct immunofluorescence allows to detect IgG and C3 bound in IC slots of epidermis.

Clinical picture
Intraepidermal vesicle or bulla, without any significant predilection – later it comes to generalisation. These changes are determined by the acantholysis. The blisters have a tight cover and pure content. Later it becomes turbid, the area around is inflammated, a blister bursts and leads to the erosion. The erosion gets wet, dirty brown greasy crusts appear on its surface and then begin to smell badly. The skin around is also eroded - it looks normal, but it's possible to wipe it away with mild pressure up to the erosion – Nikolsky's sign, acantholysis also determines indirect Nikolsky's sign. Pruritus does not occure in the skin, erosion burns and hurts, epithelialization is slow and in the end it heals without scar formation. Later the new blisters are formed comming in attacks. More malignant forms can cause death. Nowadays patients die rather from the consequences of treatment (cachexia or intercurrent illness) than from the disease itself. Disease on the mucous membranes has the similar course and clinical picture – salivation, foetor ex ore. The illness occurs mostly in patients aged from 40 to 70 years.

Differential diagnosis
Mainly bullous pemphigoid and dermatitis herpetiformis Duhring.

Histology
Intraepidermal blister with acantholysis and acanthotic cells (pyknosis).

Prognosis
All forms are severe, with unfavorable prognosis, patients usually die after several years – cachexia, infection.

Treatment
Treatment is only symptomatic - corticosteroids, ACTH, immunosuppressive drugs, ATB.

Pemphigus vegetans
Is milder and more chronic, can transform into vulgaris and regress, is intertriginous, bottoms of the blisters spread in red vegetations covered by the smelly detritus, merge in the verrucous areas, often secondarily infected by the yeasts.

Pempfigus foliaceus
Affects the whole body skin in middle-age people in form of the exfoliative erythroderma. Acantholysis is subcorneal. ;

Wet and smelly crustasquamas are formed in the seborrheic predilection, mucosa is usually not affected, itchy.

Pemphigus erythematosus
It's a bounded and milder form of the pemphigus which is characterized by the formation of symmetric, numular deposits in the seborrheic localization covered by firmly adhering crustasquamas.

Pemphigus herpetiformis
Reminds rather dermatitis herpetiformis or pemphigoid, products strongly itching blisters.

Related articles

 * Autoimmunity
 * Skin
 * Pemphigoid