Bullous Pemphigoid

Overview

Bullous pemphigoid is a rare skin condition characterized by large, fluid-filled blisters that appear on areas of skin that flex, like the lower abdomen, upper thighs, or armpits. It mainly affects older adults and can be localized or widespread. The blisters often form on areas where the skin folds, such as the armpits, groin, or abdomen. In some cases, they may also occur on mucous membranes in the mouth, tongue, throat, esophagus, or eyes.

The condition is caused by the immune system attacking a thin layer of tissue beneath the outer layer of skin, though the exact reason for this immune response is not known. It can sometimes be triggered by certain medications.

In many cases, bullous pemphigoid resolves on its own within a few months, but it may take up to five years to completely heal. Treatment usually involves using corticosteroid medications like prednisone and other drugs that suppress the immune system. Managing the condition helps in healing the blisters and reducing itching. Bullous pemphigoid can be life-threatening, especially for older individuals in poor health.

Symptoms

Bullous pemphigoid usually manifests in arms, legs, abdomen, groin, and mouth. Blisters usually occur along the skin’s creases. They are normally not uncomfortable, but they might rupture and create a painful sore or ulcer.

Common signs and symptoms may include:

• Eczema or a rash resembling a hive.
• Skin itchiness weeks or months before blisters form.
• The skin around the blisters may appear normal or discolored, such as red, purple, brown, or a shade darker than the natural skin color.
• Large blisters that do not rupture easily when touched, usually near creases or wrinkles in the skin.
• Minor blisters or sores on the lips, tongue, or other mucous membranes such as benign mucous membrane pemphigoid.
• Blisters may be clear or may contain blood.

If you experience unexplained blistering, blisters on your eyes, or any signs of infection, it is crucial to seek medical attention from your doctor promptly.

Causes

Blisters occur due to an immune system malfunction, where the body produces antibodies against its own tissues. Specifically, in bullous pemphigoid, the immune system targets the fibers connecting the outer layer of skin (epidermis) to the next layer (dermis), resulting in inflammation, blisters, and itching. The exact reasons for this antibody response remain unclear, but it leads to the characteristic symptoms of bullous pemphigoid.

Bullous pemphigoid typically emerges without a clear pattern, with no specific factors directly contributing to its onset. However, certain triggers have been identified in some cases, including:

• Medications: Some prescription drugs like etanercept, sulfasalazine, furosemide, and penicillin have been linked to triggering bullous pemphigoid.
• Light and radiation: Treatment with ultraviolet light for certain skin conditions or radiation therapy for cancer has been known to potentially trigger bullous pemphigoid.
• Medical conditions: Certain disorders like psoriasis, lichen planus, diabetes, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis have been associated with an increased risk of developing bullous pemphigoid.

Risk factors

Bullous pemphigoid affects both males and women. It also commonly affects people over the age of 60, however it can occasionally afflict younger people. The risk of developing the condition increases as the person gets older.

People with neurological diseases such as dementia, Parkinson’s disease, stroke, epilepsy, and multiple sclerosis (MS) are also more prone to develop bullous pemphigoid.