Bullous Diseases
Bullous diseases, or pemphigus, are characterized by large blisters, or bullae, on the skin. These fluid-filled lesions are caused by inflammation in or under the surface layer of skin, or epidermis.
The two diseases most commonly referred to as bullous diseases are pemphigus vulgaris and bullous pemphigoid, both autoimmune disorders, although blisters may occur in a variety of other dermatological disorders, including: contact dermatitis, erythema, erythroderma, impetigo, and staphylococcal scalded skin syndrome. Blisters may also be symptoms of certain viral or bacterial infections and some automimmune diseases.
Causes of Bullous Diseases
The causes of bullous diseases are unclear, although some small percentage of cases may be triggered by certain medications. It is also believed that radiation therapy or ultraviolet light therapy may sometimes precipitate outbreaks. Researchers are investigating other possible causes of bullous diseases. These diseases are more common in patients who suffer from other autoimmune disorders.
Symptoms of Bullous Diseases
Apart from the itching and often painful sores, and the scarring that may result as these lesions heal, bullous diseases may result in difficulty with everyday functions, depending on where the blisters erupt. The mouth can be affected, resulting in problems with eating, drinking , speaking and toothbrushing. Since sores can sometimes appear on the mucous membranes, the consequences can be terribly painful, resulting in difficulty with everyday activities.
Types of Bullous Diseases
Pemphigus Vulgaris
Pemphigus vulgaris is the most common type of blistering disease, occurring primarily in individuals between 30 and 60 years of age. The disease most often first appears as blisters around the mouth and then spreads to other skin tissue. Pemphigus vulgaris is characterized by large blisters that burst easily so an individual with this disorder usually presents with ruptured blisters or scabs. In pemphigus vulgaris the body attacks the epidermis on a superficial level. Because of the increased possibility of systemic infection, pemphigus vulgaris has some increased risk of mortality associated with it.
Bullous Pemphigoid
In bullous pemphigoid, the antibodies attack the lower membrane of the epidermis, so the lesions are subepidermal and less prone to rupture. A patient with this disorder is more likely to present with intact blisters than ruptured bullae or scabs. Bullous pemphigoid does not usually begin in the mouth as pemphigus vulgaris does. In bullous pemphigoid, blisters in the region of the mouth are rare. Instead, lesions usually appear under the arms, at the backs of the knees, behind the elbows and in the groin.
Bullous pemphigoid is a less serious disease than pemphigus vulgaris since its blisters do not rupture to allow bacterial invasion. Nonetheless, it may involve a large portion of skin surface and may be extremely uncomfortable. The first symptom of bullous pemphigoid is usually itching, followed by the appearance of large blisters or hives.
Diagnosis
Diagnosis of bullous diseases may not be simple. While bullous pemphigoid is usually recognizable because of its large blisters, it may be difficult to distinguish it from pemphigus vulgaris or even from other blistering conditions like poison ivy. A skin biopsy is usually necessary for a definitive diagnosis. A blood sample to measure antibodies levels may also be required. A special test called Nikolsky's sign in which the top layers of the skin slip away from the lower layers when slightly rubbed may also be performed.
Treatment of Bullous Diseases
Treatment of bullous diseases is generally focused on symptom control. In order to reduce inflammation and itching, high doses of corcorticosteroids are frequently prescribed. The medication may be injected, taken orally, or applied as a topical cream. Other medications that may be used to treat severe cases of these disorders are immunosuppressants, such as immunoglobin, which may be administered intravenously.
More recently, biologic medications have been prescribed as treatment for bullous diseases. Antibiotics are frequently prescribed for resulting infections. A blood purification procedure called plasmapheresis may also be employed. When bullous diseases are severe, hospitalization may be temporarily required.
Recovery from Bullous Diseases
Even without treatment, many cases of bullous diseases resolve with 6 years. Treatments may result in remission, but it is possible for bullous diseases to recur at a later date.