Pemphigus

This describes a group of disorders in which the skin splits at different levels within the epidermis. There is dissolution of the intracellular cement which holds the epidermal keratinocytes together caused by an antibody that acts directly on these intracellular proteins. The epidermal cells then no longer hold together and flaccid, easily broken blisters appear. The most common type of pemphigus (pemphigus vulgaris) is still very rare (probably around 1 in a million), although it is seen more commonly in Ashkenazy Jews and Indians. It tends to affect a younger age group than bullous pemphigoid, striking 50- to 60-year olds.

Because the skin splits within the epidermis, the blisters have very thin roofs to them and the skin becomes extensively eroded very quickly leading to fluid and protein loss and the potential for severe skin infections. In addition to this, the mouth and other mucosal surfaces are commonly affected with the consequent difficulty with eating and drinking. Patients with pemphigus can quickly become very unwell.

Treatment and nursing care are similar to bullous pemphigoid, being based on immunosuppression through high dose oral steroids. The patient is likely to be hospitalised and nursing care should include monitoring for the effects of high steroid doses along with fluid balance and care of the skin erosions (blisters will usually burst themselves as they are so fragile). In addition oral care is likely to be needed.