Pemphigus

Overview

Pemphigus is an autoimmune disorder marked by the emergence of sores, blisters, or fluid-filled lesions on the skin and mucous membranes, impacting areas such as the eyes, nose, mouth, throat, and genitals. These blisters are fragile and can easily rupture, causing painful sores that may spread and risk infection if left untreated. Although it shares similarities with other autoimmune blistering diseases like bullous pemphigoid, lupus erythematosus, and Hailey-Hailey disease, pemphigus is distinct in being non-contagious and requires ongoing medical management throughout a person’s life.

There are multiple forms of pemphigus, each classified by the location and cause of lesion development:

• Pemphigus vulgaris: The most prevalent form in the U.S., where blisters consistently involve the mouth, potentially extending to the skin and other mucous areas. Lesions form in the superficial skin layers, causing painful, slow-healing red and white fluid-filled blisters or sores, particularly around the groin and legs.
• Pemphigus vegetans: Similar to pemphigus vulgaris, but with thicker lesions that tend to occur in body areas with skin folds, such as the groin and armpit.
• Drug-induced pemphigus: Triggered by certain medications, including antibiotics and blood pressure drugs, where blisters can surface months after starting the medication.
• Pemphigus erythematosus (senear-usher syndrome): An overlap syndrome with lupus, this type causes blistering on the upper back, chest, cheeks, and scalp, with lesions often appearing red and scaly.
• Pemphigus foliaceus: Characterized by blisters on the scalp, face, neck, and back, with rare mouth involvement. This form impacts the outermost skin layer, leading to small blisters that may open and become crusty, spreading over large skin areas.
• Endemic pemphigus (fogo selvagem): A variant of pemphigus foliaceus more common in South and Central America, especially Brazil.
• Paraneoplastic pemphigus: The rarest type, associated with cancer, presents severe mouth blisters. A diagnosis prompts an investigation for underlying cancer.

Symptoms

Blisters on the skin and mucous membranes are an indicator of pemphigus. Blisters burst easily, producing exposed wounds that might leak fluid and get infected.

The signs and symptoms of two common types of pemphigus are as follows:

• Pemphigus vulgaris: Blisters on your lips, skin, or the genital area mucous membranes are typically the first signs of such a condition. Although they don’t itch, the blisters are usually painful. Harder to chew and consume when you have blisters in your mouth or throat.
• Pemphigus foliaceus: Blisters of this kind appear on the shoulders, back, and chest. Usually, the blisters itch more than they hurt. Mouth blisters are not brought on by Pemphigus foliaceus.

Sores and blisters are prone to infection. An infection’s skin symptoms include:

• The blister is filled with white or yellow pus, which leaks out if it bursts.
• A painful or burning feeling upon touching.
• If the blister bursts open, a yellow crust develops on it.
• Skin that does not heal.
• The blister and its surrounding region swells or enlarge.

If you get blisters on your skin or inside your mouth that won’t go away, it is recommended to see a healthcare provider.

Causes

Pemphigus is an autoimmune disorder where the body’s immune system mistakenly targets healthy cells, leading to blisters or sores on the skin. Although the precise cause remains unknown, it is believed that a combination of genetic predispositions and environmental factors contributes to the condition. Research indicates that certain HLA genes, which play a crucial role in the immune system, may make individuals more susceptible to pemphigus. Additionally, in rare instances, the use of specific medications, such as penicillin, piroxicam, and some blood pressure drugs, has been linked to triggering the disorder.

Risk factors

The following may increase the risk of pemphigus:

• Age: Pemphigus can affect anyone, but people who are at middle-aged or older are at risk.
• Race: People who are Jewish or of Middle Eastern heritage seem to have higher rates of the disease