The body mistakenly sends antibodies to bind to cells in the skin. These antibodies trigger a chain reaction that separates the bottom layer of cells from above layers.
The condition can affect people of any age but tends to impact older adults. Pemphigoid can also develop during pregnancy or from the use of certain kinds of medication and therapies.
While there is currently no cure for pemphigoid, several treatment options exist.
Most forms of pemphigoid cause skin rashes and blistering. People with pemphigoid usually experience symptoms periodically in between times of remission, often for months to years.
The location, extent, and timing of these symptoms vary among individuals and the types of pemphigoid experienced.
Bullous pemphigoid is more likely to impact older adults and may be caused by medications.
Image credit: Jim France, 2013
Bullous pemphigoid tends to cause blistering on areas, such as the lower torso, groin, armpits, inner thighs, soles, and palms.
The condition often presents as extremely itchy patches of raised, irritated skin along with blisters that do not burst readily. Blisters filled with clear or bloody fluid can range in width from a few millimeters to centimeters.
The skin surrounding blisters can appear unaffected or red. While symptoms are often painful, scarring does not usually occur.
Most people with bullous pemphigoid experience a flare-up of symptoms followed by periods without symptoms. The condition can persist for years.
An estimated 10 to 40 percent of those with bullous pemphigoid also experience blistering on the mucous membranes.
Each year, around 7 to 10 people out of every 1 million develop pemphigoid in the United States.
While rare, pemphigoid is the leading cause of blistering disorders in those over 65. The chances of developing the condition rise drastically after the age of 70.
Some therapies and medications can cause bullous pemphigoid. Other specific health conditions are thought to increase the likelihood of developing the condition.
Common risk factors include:
- skin injury, especially severe wounds, infections, and burns
- ultraviolet light, especially UV-based therapies
- ionizing radiation, especially radiation-based therapies
- neurological conditions, such as Parkinson's or dementia
- Grave's disease (thyroid)
Cases of cicatricial pemphigoid (CP), also known as mucous membrane pemphigoid, often involve blistering exclusively on the mucous membranes.
Blisters often become significant ulcers that lead to a loss of skin and subsequent scarring. The extent of scarring from severe cases can result in disfigurement.
Many people with CP initially experience blistering in the mouth before the CP moves onto other mucosal linings, such as the eyes and nose. People usually experience the condition for the first time between the ages of 40 and 70.
Women are thought to be two times more likely than men to experience CP. Those with a weakened immune system also seem to be at a higher risk of developing the condition.
Parts of the body commonly affected include:
- esophagus (swallowing muscle)
In some cases, the scalp, face, and neck may also be affected. Skin blistering is thought to occur in 25 to 30 percent of those with CP.
Cases of CP almost always require medical attention. Blistering in the mouth can make eating difficult. If severe, it can lead to malnourishment or weight loss. Blistering and scarring of the mucous membranes of the eye can lead to vision impairment or loss.
This form develops during pregnancy, causing blistering and a very itchy skin rash on the upper body.
Papules tend to develop first, appearing as hive-like sores on the abdomen, especially around the bellybutton. The sores then move outward, affecting the trunk and limbs.
After a few weeks, blisters often form in a circular pattern next to or within patches of papules. Scarring does not typically occur unless accompanied by infection.
In less than 5 percent of all cases, the condition can be passed from the mother to child in the womb.
Pemphigoid gestationis develops suddenly during the late stages of pregnancy. The condition can occur at any point during gestation, however, and may flare up during or directly after delivery.
For most women, the risk of developing pemphigoid gestationis is small, affecting around 1 in 50,000 pregnancies.
The condition occurs most commonly in white women and those who have had multiple previous pregnancies or have used oral contraception. Pemphigoid gestationis is also much more common in women with additional autoimmune conditions.
If characteristic blisters are present, doctors will often diagnose bullous pemphigoid by taking a skin biopsy. For more unusual cases, such as those causing itchy skin rash without blisters, blood tests may also be required.
A biopsy may be ordered by a doctor to diagnose bullous pemphigoid and pemphigoid gestationis
Bullous pemphigoid can be distinguished from other blister-causing skin conditions by the presence of certain factors, such as:
- head and neck are unaffected
- little to no mucosal membrane symptoms
- little to no pitting or scarring
A diagnosis of CP is typically made using a combination of patient history, physical examination, and a biopsy of blisters or affected mucosal tissue.
Pemphigoid gestationis is usually diagnosed using a skin biopsy. Doctors can tell the condition apart from other disorders by checking for antibodies in both skin and blood samples.
Thyroid testing is often frequently done to distinguish pemphigoid gestationis from other autoimmune disorders that can cause similar symptoms, such as Grave's disease.
Outlook and treatment
Doctors typically recommend steroid-based medications as the first line of treatment for people with severe symptoms. Additional medications are often used to manage symptoms further or treat complications.
Other treatment options for pemphigoid include:
- IVIG therapy
- skin emollients or moisturizers to reduce itchiness
- pain-relief medications, such as Tylenol or aspirin
- anti-inflammatory drugs, such as methotrexate
- antibiotics if infection occurs
- drugs to treat steroid side effects, such as hypertension, osteoporosis, and gastritis
- immunosuppressants, such as mycophenolate mofetil, rituximab, and azathioprine (to minimize steroids)
Hospitalization or professional wound dressing is usually necessary for people with infected or scarring blisters.
A doctor will likely recommend continual monitoring, as cases of pemphigoid often require weeks to years of treatment before the symptoms resolve entirely. Recurrences are also extremely common.
Many of the medications used to treat types of pemphigoid are the same. However, the specifics of individual treatment plans depend on the type, severity, and extent of symptoms.
Treatment and outlook for bullous pemphigoid
Steroid-based medications are often used to help treat severe or persistent symptoms of bullous pemphigoid. Doctors try to keep dosage levels as low as possible and stop prescribing them as soon as symptoms are gone.
A common goal for treatment plans is 5 to 10 milligrams of prednisone daily. It often takes several weeks of steroid use to reduce symptoms and can take months to years for the symptoms to resolve.
The symptoms sometimes resolve on their own. In older adults or those with weakened immune systems, however, it can cause serious health complications. If blisters burst and become infected, life-threatening blood infections can occur.
Even with medical treatment, 1-year mortality rates for severe cases of bullous pemphigoid may be as high as 25 to 30 percent. Some research also suggests a link between bullous pemphigoid and the recurrence of existing cancers.
Treatment and outlook for cicatricial pemphigoid
Depending on the location and extent of symptoms, doctors typically treat cases of CP with some form of steroid-based medication. The most commonly recommended medications and home remedies include:
Cicatricial pemphigoid is commonly treated with a topical steroid ointment.
- topical steroid ointments, creams, rinses, or washes
- topical ciclosporin rinse
- corticosteroid eye drops
- steroids injected directly into the lesion
- regular dental hygiene, including routine dental exams
- eating soft or liquid foods to avoid further irritation of blisters and associated pain
- use of lubricants or emollients on genital on skin blisters
If lesions become severe, surgery may be required.
Many people with CP need long-term monitoring and management to prevent a recurrence of symptoms. Symptoms are often slow to respond to medication and may never fully resolve.
Treatment and outlook for pemphigoid gestationis
Most cases of pemphigoid gestationis are mild and do not require direct medical treatment. Symptoms tend to resolve on their own within the first few weeks to months after the baby is born.
Doctors may prescribe topical steroids if mild cases present troublesome or persistent symptoms. Antihistamines are also commonly used to reduce itchiness.
For more severe cases, doctors may prescribe oral steroids. Additional medications, such as antibiotics, may be appropriate if severe symptoms persist after the baby is born or complications, such as infection, occur.