Introduction: Bullous pemphigoid is an autoimmune condition causing sub epidermal blistering of the skin.
If untreated, it can persist for months or years, with periods of spontaneous remissions and exacerbation. The disease can be fatal, particularly in patients who are debilitated.
Bullous pemphigoid is most common in people older than age 60.
Pathophysiology: The bullae are formed by an immune reaction, initiated by the formation of IgG autoantibodies targeting Dystonin, also called Bullous Pemphigoid Antigen. It can also occur secondary to the development of antibodies against hemidesmosomal proteins BP180 and BP230.
Clinical features: The primary feature of bullous pemphigoid is the appearance of large blisters that don't easily rupture when touched. The fluid inside the blisters is usually clear but may contain some blood. The skin around the blisters may appear normal, reddish or darker than usual. Some people with bullous pemphigoid develop an eczema or hive-like rash rather than blisters.
In most cases, the blisters appear on the lower abdomen, groin, upper thighs and arms. Blisters are often located along creases or folds in the skin, such as the skin on the inner side of a joint.
The affected areas of skin can be very itchy.