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:
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Medications: Prescription drugs may trigger pemphigoid, including Alogliptin, Penicillin, Etanercept, Sulfasalazine, and Furosemide.
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Light and Radiation: Ultraviolet light therapy for skin diseases and radiation therapy to treat cancer may trigger pemphigoid.
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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.
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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.
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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.
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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:
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Itchy welts that resemble hives or multiple painful blisters (bullae).
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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.
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The skin around the blisters may appear normal, reddish, purplish, brownish, or slightly darker than normal skin tone.
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The fluid inside the blisters may be transparent or contain some blood.
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Small blisters or sores can form in mucous membranes, such as the mouth, tongue, throat, esophagus, or eyes (benign mucous membrane pemphigoid).
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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:
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Older adults. However, younger people can also get involved by pemphigoid.
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People with neurological disorders include dementia, Parkinson's, stroke, epilepsy, and multiple sclerosis.
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The risk is more in people with psoriasis that can get aggravated by treatment of psoriasis with phototherapy.
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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:
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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.
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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:
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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.
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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.
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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.
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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.
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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:
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Avoid prolonged sun exposure and wear sunscreen outdoors.
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Protect skin by wearing soft, loose-fitting cotton clothes.
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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.
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Avoid standing or walking for extended periods of having pemphigoid on the feet.
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Limit activities if blisters are on the feet and hands, making walking or performing daily activities difficult.
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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:
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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.
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Avoid eating hot foods. Let the food cool down before eating.
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Avoid acidic food items, such as hot peppers, salsa, citrus fruits, and tomatoes.
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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:
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Unexplained blistering. Early diagnosis and treatment can help to manage this condition faster.
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Signs of infection. If the blisters pop and become infected, there is a risk of developing sepsis, a life-threatening condition.
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Blisters in the eyes.
How Can Pemphigoid Be Prevented?
It still needs to be clarified how to prevent bullous pemphigoid.
Conclusion
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.
