Pemphigus
Definition
A group of serious diseases of the skin characterized by bullae of various sizes on apparently normal skin and mucous membranes.
It is an autoimmune disease involving immunoglobulin G.
Pathology
Pemphigus is directed against a specific cell-surface antigen in epidermal cells.
Blisters form because of the antigen-antibody reaction
The level of serum antibody is predictive of disease severity
Genetic factors play a role.
Usually occurs in men and women in middle and late adulthood
May be associated with penicillins and captopril and with myasthenia gravis
Clinical Manifestations
Irregularly shaped erosions appear in the oral cavity
Painful and bleed easily and heal slowly
Skin lesions : bullae that enlarge, rupture and leave large painful eroded areas accompanied by crusting and oozing
Offensive odor
Adjucent skin is blistered and peels off on minimal pressure (Nikolsky's sign)
Eventually huge areas of the body are involved
Secondary bacterial infection occurs
Complications
Secondary infection by bacteria
Fluid and electrolyte imbalance due to loss of fluid and protein as the bullae rupture
Hypoalbuminemia
Management
Corticosteroids in high doses until remission
Life long therapy may be needed.
Evaluate body weight, BP, blood gulcose levels and fluid balance.
Immunosuppressive agents ( azathioprine, cyclophosphamide, gold) may be needed
In life threatening cases plasmapheresis (plasma exchange) to decrease antibody levels
