Pemphigus: Symptoms, Types and Treatment

PEMPHIGUS

DEFINITION: "A very serious autoimmune disease caused by a disorder of cohesion between intraepidermal cells, resulting in blisters and painful erosions. It is a chronic, relapsing dermatosis, mainly affecting older people, with changes on the skin and mucous membranes. A very severe general condition, with periods of exacerbation alternating with remissions. There is no definitive cure; the disease is capricious and resistant."

TYPES:

• Pemphigus vulgaris;
• Pemphigus vegetans;
• Pemphigus foliaceus;
• Pemphigus erythematosus (Brasiliensis);
• Pemphigus chronicus familiaris benignus;
• Pemphigus herpetiformis;
• Grover syndrome.

Pemphigus vulgaris

Diagnostic criteria:

• Filled blisters with a thin roof, on unchanged skin
• Painful erosions
• Face, trunk, flexor surfaces
• Affected mucous membranes, especially the oral cavity
• Positive Nikolsky sign
• Etiopathogenesis: An autoimmune disease caused by acantholysis (splitting of intercellular bridges of epidermal cells), as a result of the action of autoantibodies (IgG and C3) against the autoantigen (desmosomes)
• Can be demonstrated by direct and indirect immunofluorescence.
• Clinical picture: The disease often begins with changes on the oral mucosa in the form of erosions covered by whitish fibrinoid deposits. Similar changes may localize to the trachea, larynx, esophagus and stomach. Skin changes appear suddenly: blisters the size of a chicken egg burst quickly, leaving painful reddish erosions that heal without scarring.
• Blisters appear on UNCHANGED skin and do not itch or hurt. They appear in bursts at various time intervals.

• Therapy:
• Systemic use of high doses of corticosteroids (severe forms 120 to 200 mg; moderately severe 80 to 120 mg), in combination with immunosuppressants (cyclophosphamide 50 to 100 mg daily or azathioprine 100 to 200 mg).
• Therapy lasts 4 to 8 weeks, after which corticosteroids are reduced every 5-6 days by 5 to 10 mg to a maintenance dose of 5-15 mg prednisolone daily. The immunosuppressive effect is achieved after 3-6 weeks, followed by dose reduction. Cyclosporine 5-6 mg/kg/day; plasmapheresis; local antibiotics and epithelializing agents.

Pemphigus vegetans

• A form of pemphigus in which vegetations appear at the base of erosions. Pemphigus vegetans Neumann: vegetations distributed intertriginously, preceded by bullae and vesicles.

Pemphigus foliaceus

• A form of pemphigus with barely pronounced blistering, dominated by moist, scaly deposits.

Pemphigus erythematosus

• Pemphigus seborrhoides; Senear-Usher syndrome.
• A combination of foliaceus with the simultaneous presence of lupus erythematosus.
• On seborrheic areas (scalp, face, back) persistent erythema appears with weak skin infiltration; focal erosions, similar to foliaceus.

PEMPHIGUS CHRONICUS FAMILIARIS BENIGNUS

• Hailey-Hailey disease; an autosomal dominant hereditary disease of variable gene penetrance, HLA B8.
• Therapy - Medium-high doses of corticosteroids, 60-80 mg daily; local antibiotic application; etretinate, isotretinoin. Prognosis is good. Possible complications: secondary bacterial infection.