Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.
In most cases of bullous pemphigoid, no clear precipitating factors are identified. Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy. Onset of bullous pemphigoid has also been associated with certain drugs, including furosemide, and other nonsteroidal anti-inflammatory agents, captopril,penicillamine, and antibiotics.
The most common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common. The blisters have certain characteristics, regardless of where on the body they form: they are often preceded by a red rash, they are large and filled with fluid that is usually clear, but may contain some blood they are thick and do not rupture easily, the skin around the blisters may appear normal or slightly red or dark, ruptured blisters are usually sensitive and painful.
Pemphigoid cannot be cured, but treatments are usually very successful at relieving symptoms. Corticosteroids, either in pill or topical form, will likely be the first treatment your doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause serious side effects, especially from long-term use, so your doctor will take you off of the corticosteroids after the blistering clears up. Another treatment option is to take medication that suppresses your immune system, often in conjunction with the corticosteroids. Immunosuppressants help, but they also put you at risk for other infections. Certain antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.
We conducted a retrospective cohort study of all newly diagnosed patients with BP seen at the National Skin Centre from 1 April 2004 to 31 December 2009. Demographic and clinical data on comorbidities and treatment were recorded. Mortality information was obtained from the National Registry of Diseases. In total 359 patients were included in our study. The 1-, 2-, 3-year mortality rates were 26 7%, 38 4% and 45 7%, respectively. The 3-year standardized mortality risk for patients with BP was 2 74 (95% confidence interval 2 34-3 19) times higher than for the age- and sex-matched general population. Parkinson disease, heart failure and chronic renal disease were associated with increased mortality, while combination treatment with low-to-moderate-dose corticoste-roids and immunomodulatory agents such as doxycycline and/or nicotinamide was associated with lower mortality. Overall, infections were the most common cause of death (59 8%), with the main causes of death being pneumonia (42 7%), cardiovascular disease (14 6%) and stroke (11 6%). A total of 359 patients were included in our study. The 1-, 2-, 3-year mortality rates were 26.7%, 38.4%, and 45.7%, respectively. The 3-year standardized mortality risk for BP patients was 2.74 (95% CI: 2.34 - 3.19) times compared to the age- and sex-matched general population. Parkinson's disease, heart failure and chronic renal disease were associated with increased mortality, while combination treatment with low to moderate dose corticosteroid and immunomodulatory agents such as doxycycline and/or nicotinamide was associated with lower mortality. Overall, infections were the commonest cause of death (59.8%), with main causes of death being pneumonia (42.7%), cardiovascular disease (14.6%) and stroke (11.6%).This study confirmed an increased 3-year mortality rate for BP patients in Singapore.