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Pemphigoid - Causes, Symptoms, Diagnosis, Risk Factors, and Management

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Pemphigoid is a rare autoimmune skin disorder that causes large, itchy, fluid-filled blisters. The below article details the same.

Medically reviewed by

Dr. Sandhya Narayanan Kutty

Published At January 6, 2023
Reviewed AtJune 27, 2023

What Is Pemphigoid?

Pemphigoid is a rare autoimmune skin condition that causes large, itchy, fluid-filled, or hive-like welts blistering on the skin. The disease may involve a small body area or may be widespread. Blisters can develop anywhere, but most commonly on flexural areas of the skin, like the lower abdomen, under the armpits, or upper thighs. In some cases, blisters can also appear on mucous membranes, such as the mouth, tongue, throat, esophagus, or eyes. Pemphigoid is common in older adults. Pemphigoid is not contagious and cannot spread from one person to another through skin-to-skin contact. However, pemphigoid can be fatal, especially for older people already in poor health or not seeking treatment.

What Causes Pemphigus?

Pemphigus is an autoimmune disease due to a malfunction in the immune system. The body's immune system produces antibodies to fight against bacteria, viruses, or potentially toxic substances. However, in pemphigoid, the immune system makes antibodies to the fibers joining the outer layer (epidermis) and the next layer of skin (dermis). These antibodies trigger inflammation that causes painful blistering eruptions on the skin.

Triggering Factors:

Pemphigoid usually occurs randomly, with no apparent factors contributing to disease onset. However, some cases of pemphigoid may be triggered by:

  • Medications: Prescription drugs may trigger pemphigoid, including Alogliptin, Penicillin, Etanercept, Sulfasalazine, and Furosemide.

  • Light and Radiation: Ultraviolet light therapy for skin diseases and radiation therapy to treat cancer may trigger pemphigoid.

  • Medical Conditions: Some disorders that can trigger pemphigoid include diabetes, psoriasis, lichen planus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis.

What Are the Three Types of Pemphigoid?

Pemphigoid is of three types. These types differ in the locations of blisters and when these blisters occur.

  • Bullous Pemphigoid: It is the most common of all types of pemphigoid. In bullous pemphigoid, the blisters commonly appear on flexural areas of the skin, like the lower abdomen, under the armpits, or upper thighs.

  • Cicatricial Pemphigoid: Also called mucous membrane pemphigoid, it refers to blisters on the mucous membranes, such as the mouth, eyes, nose, throat, and genitals. The rashes and blisters may form in one of these areas and spread to the others if left untreated. If left untreated in the eyes, it may cause scarring, resulting in blindness.

  • Pemphigoid Gestationis: This form of pemphigoid occurs when blistering occurs during or immediately after pregnancy. The blisters typically appear during the second or third trimester but can also happen at any time during pregnancy or up to six weeks after delivery. The commonly affected sites are the arms, legs, and abdomen.

What Are the Symptoms of Pemphigoid?

The common signs and symptoms of pemphigoid may include:

  • Itchy welts that resemble hives or multiple painful blisters (bullae).

  • Large blisters typically appear along the folds or creases of the skin. These blisters are usually not painful but may rupture and become painful sores.

  • The skin around the blisters may appear normal, reddish, purplish, brownish, or slightly darker than normal skin tone.

  • The fluid inside the blisters may be transparent or contain some blood.

  • Small blisters or sores can form in mucous membranes, such as the mouth, tongue, throat, esophagus, or eyes (benign mucous membrane pemphigoid).

  • On rare occasions, itchy welts and blisters may result in scarring.

What Are the Risk Factors of Pemphigoid?

Pemphigoid usually affects men and women equally. However, some people are at an increased risk of getting pemphigoid, including:

  • Older adults. However, younger people can also get involved by pemphigoid.

  • People with neurological disorders include dementia, Parkinson's, stroke, epilepsy, and multiple sclerosis.

  • The risk is more in people with psoriasis that can get aggravated by treatment of psoriasis with phototherapy.

  • People on specific drugs, having an injury, or skin infections are also at particular risk of pemphigoid.

How Is Pemphigoid Diagnosed?

The healthcare provider usually begins diagnosis by examining the affected areas. Then, the doctor may prescribe the following tests to confirm their diagnosis and prescribe the proper treatment:

  • Blood Tests: The healthcare provider can draw a blood sample from a vein in the arm using a small needle and send the blood sample to a laboratory for testing.

  • Skin Biopsy: The healthcare provider may remove a small sample of the affected skin and send the sample for laboratory testing to confirm the diagnosis.

How Is Pemphigoid Treated?

Pharmacological Treatment

Treatment usually focuses on healing the skin, soothing itching, and minimizing the side effects of medications. In addition, the doctor may prescribe one or a combination of the following drugs:

  • Topical Corticosteroids: The best treatments for mild cases of pemphigoid are topical corticosteroid creams or ointments that can be rubbed directly on the affected areas.

  • Oral Corticosteroids: Prednisone, which comes in pill form, is the most common treatment for moderate-to-severe cases of pemphigoid. However, the long-term use of oral corticosteroids can increase the risk of weak bones, diabetes, high blood pressure, high cholesterol, and infection.

  • Steroid-Sparing Drugs: These drugs work by inhibiting the production of the body's disease-fighting white blood cells. Examples of steroid-sparing drugs include Dapsone, Azathioprine, Mycophenolate mofetil, Azathioprine, Methotrexate, and Chlorambucil.

  • Antibiotics: The healthcare provider may prescribe oral Tetracycline or Doxycycline pills to people who cannot take corticosteroids or other immunomodulatory agents. These drugs help reduce inflammation and infection.

  • Rituximab or IVIG infusions: The doctor may recommend Rituximab or IVIG infusions for refractory cases of pemphigoid.

Home Remedies

A few self care-strategies can help manage the symptoms and make the condition more tolerable, including:

  • Avoid prolonged sun exposure and wear sunscreen outdoors.

  • Protect skin by wearing soft, loose-fitting cotton clothes.

  • Follow the doctor's advice strictly for the daily care of blisters. Thoroughly wash the sores or ulcers with antibacterial soap and water to prevent infection, apply antibiotic ointment to the affected areas, and adequately wrap them in nonadhesive bandages.

  • Avoid standing or walking for extended periods of having pemphigoid on the feet.

  • Limit activities if blisters are on the feet and hands, making walking or performing daily activities difficult.

  • Moisturize skin with creams, lotions, coconut oil, or shea butter oil.

The following tips can make symptoms more tolerable and help prevent nutrition issues if the blisters are in the mouth:

  • Avoid eating crunchy and hard foods, such as crispy chips, crusty bread, raw fruits, and vegetables, that may aggravate symptoms. Instead, include soft foods in the diet, including soups, mashed foods, and pudding.

  • Avoid eating hot foods. Let the food cool down before eating.

  • Avoid acidic food items, such as hot peppers, salsa, citrus fruits, and tomatoes.

  • Avoid drinking alcohol.

Generally, one may feel better a few days after starting treatment.

What Is the Outlook for People With Pemphigoid?

The outlook for pemphigoid is good with comprehensive treatment. People with pemphigoid generally respond well to medications. The disease often goes away after a few years of treatment. However, pemphigoid may come back at any time.

When To See a Doctor?

See a doctor if the following conditions develop:

  • Unexplained blistering. Early diagnosis and treatment can help to manage this condition faster.

  • Signs of infection. If the blisters pop and become infected, there is a risk of developing sepsis, a life-threatening condition.

  • Blisters in the eyes.

How Can Pemphigoid Be Prevented?

It still needs to be clarified how to prevent bullous pemphigoid.


Pemphigoid is a rare autoimmune skin condition that causes blisters or sores. It may resolve and come back, but it generally responds well to treatment and goes away entirely after a few years. Pemphigoid can be fatal if the blisters pop and become infected. However, contact a healthcare provider immediately if a person notices unexplained blisters.

Frequently Asked Questions


What Type of Disease Is Pemphigoid?

Pemphigoid is a rare autoimmune disease of the skin or mucous membranes. Generally, the body's immune system produces antibodies to fight off infections and harmful foreign organisms in the body. But in the case of an autoimmune disease, the antibodies start to attack their healthy cells.


Can Pemphigoid Be Cured?

Pemphigoid usually goes away on its own without any treatment. However, in some cases, it can last for months or years. The treatment involves topical steroid creams, which help heal the skin and prevent new patches or blisters from appearing.


What Blood Test Checks for Pemphigoid?

The healthcare professional may recommend blood tests to confirm the diagnosis. However, a biopsy is mostly indicated in the case of pemphigoid. Some tests, such as Indirect immunofluorescence (IIF) and ELISA (enzyme-linked immunosorbent assay), are advised. These serologic tests help find the respective antibodies in the blood. The IIF test helps detect the circulating antibodies and their deposition patterns, and the ELISA helps identify and calculate the number of antibodies to antigens in pemphigoid.


Is There a Treatment for Pemphigoid in Ayurveda?

In Ayurveda, various herbs or herbs-mineral preparations can be used to treat autoimmune diseases. Some of the most commonly advised ayurvedic medicines include Arogyavardhini Rasa and Gandhaka Rasayana, which show promising results against skin disorders like pemphigoid.


Which Medicines Can Lead To Pemphigoid?

Sulfur-containing medicines such as furosemide are the most commonly associated with drug-induced pemphigoid. In addition to that, some of the following medicines can also lead to drug-induced pemphigoid, which includes -
 - Amoxicillin. 
 - Ampicillin.
 - Phenacetin. 
 - Penicillin. 
 - Penicillamine. 
 - Psoralen-ultraviolet-A light.
 - Beta-blockers.


What Treatment Is Considered the First Line of Treatment for Pemphigus?

The first line of treatment for pemphigus includes the combination of Rituximab and highly potent topical corticosteroids. The essential component of the first line of treatment for pemphigus is high doses of systemic corticosteroids, but these drugs can cause severe side effects. Therefore, these should be taken only after consulting the doctor and in the fixed dosage advised by the physician.


Can Stress Cause Pemphigoid?

Stress is not the primary cause of pemphigoid. However, it has been linked to various autoimmune diseases. In addition, stress can be one of the factors in flare-ups of pemphigoid. The stress causes the unusual activity of the antibodies and can cause more blisters.


Who Is at Risk for Pemphigus?

Pemphigus is commonly seen in people of middle age or older adults. This disease usually begins at the age of 50 to 60. The risk of getting pemphigus increases if a person has a Jewish heritage.


Is Pemphigoid Genetic?

Pemphigus does not appear to be hereditary, meaning it cannot be transferred from parents to their children. However, some people's genes put them at risk of getting pemphigoid. The development of pemphigoid can be linked to multiple factors such as genetic predisposition, ethnicity, age, and the environment.


Does Pemphigoid Cause Blindness?

Ocular cicatricial pemphigoid (OCP) is a subtype of mucous membrane pemphigoid (MMP), which include ocular manifestations that can lead to blindness. OCP is autoimmune conjunctivitis that can cause scarring (cicatrization) of the conjunctiva. It can also lead to dry eye, corneal ulcers, and neovascularization. If this condition is left untreated, it can lead to blindness.


What Areas Are Most Affected by Pemphigoid?

The most commonly affected area by pemphigoid is inside the mouth and throat, which can cause pain during swallowing. Later, the patient can have blisters on the skin, which may appear after a few months if the mouth is affected. The blisters and sores can cover the mucosal surfaces of the nose, eyes, throat, and genitals as the disease progresses.


Is Pemphigoid Life-Threatening?

The pemphigoid can be easily managed with the proper treatment, as it helps heal blisters and ease symptoms like itching. Treatment usually involves using corticosteroids such as Prednisolone. However, it can be life-threatening for older patients with poor health conditions.


Which One Is Worse, Pemphigoid or Pemphigus?

Pemphigus usually affects the outer layer of skin (epidermis), which can cause blisters that can easily rupture. However, in the case of pemphigoid, the lower layer of skin is affected between the epidermis and the dermis, creating tense blisters that do not rupture easily. Therefore, pemphigoid is more likely to cause red and itchy blisters and is more likely to leave permanent marks on the skin.
Dr. Sandhya Narayanan Kutty
Dr. Sandhya Narayanan Kutty



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