Bay Biosciences provides high quality, clinical grade, biopsy tissue samples, FFPE tissue blocks with matched cryogenically preserved K2EDTA plasma, sera (serum) and peripheral blood mononuclear cells (PBMC) biofluid samples from pemphigoid patients.
The K2EDTA plasma, sera (serum) and PBMC biofluid specimens are processed from pemphigoid patient’s peripheral whole-blood using customized collection and processing protocols.
Pemphigoid Overview
Pemphigoid is a family of rare autoimmune diseases that cause blistering and rashes on the skin and mucous membranes. 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.
This disease can affect people of any age including children but tends to impact older adults. Pemphigoid can also develop during pregnancy or from the use of certain kinds of medication and therapies.
Pemphigoid is caused by a malfunction of the immune system and results in skin rashes and blistering on the legs, arms, and abdomen.
Currently there’s no cure for pemphigoid, but there are various treatment options available to manage the disease.
Types of Pemphigoids
There are several types of pemphigoids. All types of pemphigoids are caused by the patient’s own immune system attacking healthy tissue. They appear as rashes and blisters filled with fluid. The types of pemphigoids differ in terms of where on the body the blistering occurs and when it occurs.
Bullous Pemphigoid
Bullous pemphigoid tends to cause blistering on areas, such as the lower torso, groin, armpits, inner thighs, soles, and palms.
In cases of bullous pemphigoid, the most common ones are the three types, the skin blistering happens most commonly on the arms and legs where movement occurs. This includes the areas around the joints and on the lower abdomen.
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 patients 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 the patients 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:
- Diuretics
- Etanercept
- Grave’s disease (thyroid)
- Ionizing radiation, especially radiation-based therapies
- Neurological conditions, such as Parkison’s disease or dementia
- Penicillin
- Psoriasis
- Sulfasalazine
- Skin injury, especially severe wounds, infections, and burns
- ultraviolet light, especially UV-based therapies
Cicatricial Pemphigoid
This type of pemphigoid is also known as mucous membrane pemphigoid. Cicatricial pemphigoid refers to blisters that form on the mucous membranes, which includes the following:
- Eyes
- Genitals
- Mouth
- Nose
- Throat
The most common sites affected on the body are the mouth and eyes. The rash and blistering may begin in one of these areas and spread to the other if left untreated. If it’s left untreated in the eyes, it may cause scarring, which in turn may lead to blindness.
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.
Most patients with cicatricial pemphigoid (CP) initially experience blistering in the mouth before the CP moves onto other mucosal linings, such as the eyes and nose. Usually, patients experience the disease for the first time between the ages of 40 and 70.
Women are thought to be twice more likely than men to experience cicatricial pemphigoid 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:
- Anus
- Eyes
- Esophagus (swallowing muscle)
- Genitalia
- Mouth
- Nose
- Throat
In certain cases, the scalp, face, and neck may also be affected. Skin blistering is thought to occur in 25 to 30 percent of the patients with cicatricial pemphigoid.
Most cases of cicatricial pemphigoid 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.
Pemphigoid Gestationis
In pemphigoid gestationis blistering develops during or shortly after pregnancy, causing blistering and a very itchy skin rash on the upper body. It was formerly called herpes gestationis, although it’s not related to the herpes virus.
The blistering typically develops during the second or third trimester, but may occur at any time during pregnancy, or up to six weeks after delivery. In this condition blisters develop on the patient’s arms, legs, and abdomen.
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 pemphigoid gestationis 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.
Signs and Symptoms of Pemphigoid
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:
- A red rash develops before the blisters
- Blisters are large and filled with fluid that’s usually clear, but may contain some blood
- Ruptured blisters are usually sensitive and painful
- Skin around the blisters may appear normal, or slightly red or dark
- The blisters are thick and don’t rupture easily
Diagnosis of Pemphigoid
Doctors will often diagnose bullous pemphigoid by taking a skin biopsy which involves removing small samples of skin from the affected areas.
For more unusual cases, such as those causing itchy skin rash without blisters, blood tests may also be required to prescribe the right treatment.
Samples are sent to a lab to test for the immune system antibodies characteristic of pemphigoid. These antibodies can be detected in the blood sample taken from the patient.
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
- No pitting or scarring
A diagnosis of cicatricial pemphigoid 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.
Treatment of Pemphigoid
Pemphigoid is usually treated by steroid-based medications as the first line of treatment for patients with severe symptoms. Additional medications are often used to manage symptoms further or treat additional complications.
Other treatment options for pemphigoid include:
- Anti-inflammatory drugs, such as methotrexate
- Dapsone
- Antibiotics if infection develops
- IVIG therapy
- Nicotinamide
- Immunosuppressants, such as mycophenolate mofetil, rituximab, and azathioprine (to minimize steroids)
- Pain-relief medications, such as Tylenol or aspirin
- Skin emollients or moisturizers to reduce itchiness
- Drugs to treat steroid side effects, such as hypertension, osteoporosis, and gastritis
Hospitalization or professional wound dressing is usually necessary for patients 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 go away completely. Recurrences are also extremely common.
Many of the medications used to treat types of pemphigoids are the same. However, the specifics of individual treatment plans depend on the type, severity, and extent of symptoms.
Treatment of 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 of Cicatricial Pemphigoid
Depending on the location and extent of symptoms, doctors typically treat cases of cicatricial pemphigoid with some form of steroid-based medication. The most commonly recommended medications and home remedies include the following:
- Eating soft or liquid foods to avoid further irritation of blisters and associated pain
- Topical steroid ointments, creams, rinses, or washes
- Corticosteroid eye drops
- Topical ciclosporin rinse
- Steroids injected directly into the lesion
- Regular dental hygiene, including routine dental exams
- Use of lubricants or emollients on genital on skin blisters
If lesions become severe, surgery may be required.
Many people with cicatricial pemphigoid 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 of 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.
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