Symptoms
Bullous pemphigoid typically starts off with a very itchy rash that has an appearance similar to that of hives. This rash may recur for several months. Eventually, the rash evolves to areas that form blisters that then break and leave areas of sore, open skin. The blisters are tight and hard, and the skin between blisters may be red and swollen. The whole body can be affected.
Cicatricial pemphigoid is a blistering disease that causes scarring. Ocular pemphigoid, which affects the eyes, is a common form and can be associated with a feeling of dry, gritty eyes. It can lead to blindness. If the mouth is affected, there can be sore open areas of gums, cheeks, tongue and/or palate. If the throat is involved, swallowing and talking may be affected and be life-threatening if not treated. Body and scalp sores can also appear.
Pemphigus vulgaris may start with oral ulcers that can be mistaken for the more common aphthous ulcers (“canker sores”). These are very painful and interfere with eating and talking. Another early sign are scalp sores, which are like open wounds and can be confused with a local scalp infection. Skin sores are called blisters, but these are weak and break so easily that it looks more like the skin has been scraped, resulting in red and weepy areas. About one half of people with pemphigus vulgaris will develop blisters and sores on their skin. As it is rare, it is common for the condition to progress for months or years before a diagnosis is made.
Pemphigus foliaceus is a rash that has a more scaly appearance—like eczema—rather than like blisters. The face is a common site, but it can also occur on the scalp and body. The skin is very prone to infection and sometimes will be extremely itchy or painful.
Paraneoplastic pemphigus (pemphigus that is caused by an underlying cancer) usually presents with very sore and eroded lips, pain and redness of the eyes, and a widespread rash that has areas that blister and become sore, along with other areas that are itchy and hive-like.
