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.
A retrospective chart review was performed comprising all patients with clinical and/or histopathological diagnosis of BP treated at the Dermatology Department of Pedro Ernesto University Hospital in Rio de Janeiro, Brazil from 2006 to 2012. Next, those with histopathological confirmation of BP were selected . Records of 40 patients were analyzed, 15 of which were excluded for not having conclusive histopathological data on BP. The average age of patients was 73.9 years, ranging from 38 to 92 years and regarding gender, 18 women (72%) and 7 men (28%) were studied. The age range of patients who showed the highest association of BP and ND was between 80 and 89 years. Of the 25 patients with histopathologically confirmed BP, 9 (36%) had clinical diagnosis of at least one neurological or psychiatric disorder and 9 (36%) did not have associated neurological diseases. In 7 cases (28%), no record that could confirm or rule out the investigated pathologies was found. Associated neurological diseases in these cases were: cerebrovascular accident (CVA) or stroke (44.4%), dementia (22.2%), panic disorder syndrome (11.1%), depression (11.1%) and epilepsy (11.1%). One patient had two associated diseases, stroke and migraine, and one patient had a history of two episodes of stroke prior to the diagnosis of BP. Our data also demonstrated the high prevalence of neurological disorders in patients diagnosed with BP (36%). Although bullous pemphigoid is not overtly prevalent in any race or sex, most of our cases were diagnosed in women (72%) and they were the ones with a more frequent association of BP and neurologic disease (77%). The majority of patients diagnosed with BP were in the age group of 70-79 years (36%), and 19 patients (76%) had more than 69 years, concurring with previously published data. Cordell et al published a study with 341 patients, in which the neurological diseases most often associated were dementia in 20% of cases, followed by stroke (15%). Conversely, Chen et al in a study with 3,485 patients found stroke as the most prevalent disease with 1,284 cases, followed by dementia (617 cases), Parkinson's disease (416 cases), epilepsy (201 patients) and schizophrenia (22 cases). Associated inflammatory diseases such as psoriasis (74 cases) and rheumatoid arthritis (20 cases) were also detected. Our results coincide with the latter study, in finding stroke as the most frequently associated neurological disease, followed by dementia, epilepsy, depression and panic disorder syndrome.