Pemphigoid

Overview of Pemphigoid

Pemphigoid is a rare autoimmune condition, causing lesions and rashes on the skin and mucous membranes, particularly in the lower abdomen, groin, and arm. In this condition, the antibodies (anti-BPA-2 and anti-BPA-1) are produced by the immune system that connects with the skin cells. This results in separating the lower layer from the upper layer of skin.

People at any age can suffer from this disease, even during pregnancy. Currently, Researchers cannot find any approved cure for pemphigoid but they focus on its management through different home remedies or medications.

In Pakistan, pemphigoid is a rare disease. It is not as prevalent in the Pakistani community but in the US the risk of pemphigoid is high, almost 25%-30% but 80% chance of recovery in most people.

Doctors Treating Pemphigoid

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Signs and Symptoms of Pemphigoid

The most visible symptom of pemphigoid is the formation of blisters on the arms, legs, abdomen, and mucous membranes. Along with it, hives-like skin and irritation have typically appeared. A few features of the blisters are important to identify on the patients like:

  • An individual is feeling redness on the skin before blistering
  • Large and fluid-filled blisters may enclose with blood
  • Thick blisters are not easy to rupture
  • Normal, slightly red or dark skin may be surrounded by the blisters
  • Painful ruptured blisters are usually sensitive to the skin

Types of Pemphigoid

Several types of pemphigoid differ from one another in terms of the affected areas of the body and duration of action. All types are based on the autoimmune reactions (attack the healthy tissues on their own). This also results in rashes and fluid-filled lesions visible on the skin. Pemphigoid types are as follows:

Bullous Pemphigoid:

Bullous pemphigoid is considered the most common type of all. The skin blisters are apparent on the surrounding areas of joints of arms, legs and lower abdomen. 

Cicatricial Pemphigoid:

Cicatricial pemphigoid is also known as mucous membrane pemphigoid. The blisters appear on the mucous membrane of the following areas of the body.

  • Mouth
  • Eyes
  • Nose
  • Throat
  • Genitals

The most commonly affected areas are the mouth and eyes. If these areas are not treated on time, then it may spread to other parts of the body such as the eyes left untreated then it may cause bruising and blindness of the eyes. 

Pemphigoid Gestationis:

This type of blister appears during or after pregnancy and is usually formed in the second or third trimester. These blisters may be apparent at any time of pregnancy or 6 weeks after delivery on these main parts of the body that are:

  • Arms
  • Legs
  • Abdomen

Causes of Pemphigoid

Pemphigoid begins when the immune system automatically attacks the healthy tissues present below the outer layer of the skin. This results in the separation of layers and causes painful blistering.

There is no specific stimulator for pemphigoid but in some cases, it may be caused by:

  • Medications
  • Radiation therapy
  • Ultraviolet light therapy

People who are experiencing other autoimmune disorders are at greater risk of pemphigoid. It seems to be more common in old people than in other age groups. Similarly, the disease ratio is slightly higher in women than in men.

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Risk Factors of Pemphigoid

Some of the important risk factors for pemphigoid include:

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Prevention

Following self-care strategies are necessary for minimizing the symptoms of pemphigoid:

  • Wound care
  • Limit the outdoor activities 
  • Avoid sun exposure
  • Wear loose-fitting cotton clothes 
  • Avoid eating hard and crunchy food such as chips, raw fruits, and vegetables 
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Diagnosis

A dermatologist will examine all the characteristics of blisters physically that have already been described above (pemphigoid symptoms). When a doctor is diagnosing any type of pemphigoid through the following lab tests:

  • Physical Examination: First a dermatologist asks questions to the patients to identify the cause of the blisters. After that, a doctor takes family history, and medical history and looks at the blisters physically or under the light of a microscope. 
  • Skin Biopsy: In this technique of biopsy, a small culture of skin is removed from the affected area for analysis. This helps to diagnose the level of antibodies in the body. Skin biopsy is significant for all pemphigoid types. 
  • Blood Tests: The blood test is used to identify whether any foreign particle attacks the skin or not. It is usually performed when there is no visibility of blisters.
  • Thyroid Testing: The thyroid function test is conducted if a doctor suspects that a person may have pemphigoid gestationis symptoms. Mostly its symptoms are similar to a grave disease.

Treatment of Pemphigoid | When to Consult a Doctor

The basic treatment is home remedies that a person usually tries at home. If the condition is not treated properly, then consult the doctors.

Home Remedies:

The most suitable home remedies are applied following products on the skin for the management of pemphigoid.

  • Aloe vera
  • Ginger
  • Vitamin C
  • Sunscreens usage

Medical Treatment:

The majority of dermatologists recommend different kinds of medications such as oral drugs, injectables, emollients, creams, or gels depending on the severity of pemphigoid. Steroidal drugs are used as a first line of treatment for people with severe symptoms. Other drugs are beneficial for the management of the symptoms or may treat severe complications. 

Other recommended treatment choices for pemphigoid include:

  • IVIG therapy
  • Nicotinamide
  • Dapsone
  • Skin emollients or moisturizers (reduce itchiness)
  • Pain-relief medications (Tylenol or aspirin)
  • Anti-inflammatory drugs (methotrexate)
  • Antibiotics (if infection occurs)
  • Drugs used for side effects of steroids such as hypertension, osteoporosis, and gastritis
  • Immunosuppressants (mycophenolate mofetil, rituximab, and azathioprine) 

Hospitalization or professional wound dressing may be required for those patients who have infected or bruising blisters.

Apart from this, a dermatologist will carry out continuous monitoring until and unless the patients are completely recovered from the pemphigoid disorder. It may take weeks or years, it all matters on the intensity of the disease. Relapse of the disease is extremely common.

Treatment Specific to Pemphigoid Types:

  • Treatment for Bullous Pemphigoid:

The dermatologists prescribe drugs that relieve the patients from itching, rashes, and redness with reduced side effects. They give the medications in one or a combination form. 

Corticosteroids such as prednisone are a prime oral medication for the bullous pemphigoid treatment. But long-term use may cause some side effects like weak bones, diabetes, high blood pressure, high cholesterol, and infection. Corticosteroid ointment can be applied to the affected skin and causes fewer side effects.

The next therapy is steroid drugs that inhibit the production of antibodies by controlling the white blood cells of the body. Examples are: 

  • Azathioprine (Azasan, Imuran) 
  • Mycophenolate mofetil (CellCept)

If a person has eyes or upper digestive tract signs and symptoms (as aforementioned in signs and symptoms) then the drug rituximab (Rituxan) may be suggested.

Anti-inflammatory drugs are used for treating the inflammation of the skin like methotrexate (Trexall).

Treatment for Cicatricial Pemphigoid:

The first choice of treatment is steroidal drugs such as:

  • Topical steroid ointments, creams, rinses, or washes
  • Topical ciclosporin 
  • Corticosteroid eye drops
  • Steroids injection (administer directly into the blister)
  • Lubricants or emollients on genital skin blisters

If the cicatricial pemphigoid condition becomes severe then surgery may be recommended.

Treatment for Pemphigoid Gestationis

Most people with pemphigoid gestationis have mild symptoms and do not require direct medical treatment. These symptoms tend to reduce on their own within the first few weeks to months after delivery. A dermatologist may prescribe topical steroids in severe cases. 

Antihistamines reduce the itchiness of the skin. Along with it, oral steroids or antibiotics are preferable if symptoms may persist for a prolonged time. This may cause infection or any complications to the newborn baby of the affected women.

Important Note: Do not take any medicines without a doctor’s consultation.

In case you exhibit any concerning signs and symptoms of pemphigoid, consult a medical professional as soon as possible.