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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
Diagnosis
The rare disease pemphigus can be difficult to diagnose since it can present with a number of more common conditions that can cause blisters. A dermatologist, who specializes in skin issues, may be recommended by your healthcare provider.
- Physical examination: The healthcare provider will review your medical history, and will assess your mouth and skin. Additionally, they will recommend the patient will undergo test to properly diagnose the condition.
- Skin biopsy: A blister sample is taken for this test, and it is examined under a microscope.
- Blood tests: These tests are utilized, to find and identify antibodies in your blood that are associated with pemphigus.
- Endoscopy: Your healthcare provider can recommend an endoscopy if you have pemphigus vulgaris in order to look for throat sores. An endoscope, a flexible tube, is inserted down into your throat during this procedure.
Treatment
Medications meant to prevent the production of blisters are typically used as the first line of treatment. Starting as early as feasible usually results in greater effectiveness. If your pemphigus was brought on by taking medication, discontinuing the use of the medication may be enough to treat it.
- Medications: Depending on the kind and severity of your pemphigus and if you have any other medical issues, you may use any or all of the following prescription medications:
- Corticosteroids: Corticosteroid cream may be effective to manage minor cases of the condition in certain individuals. For others, oral corticosteroids like prednisone tablets serve as the cornerstone of their care.
- Immunosuppressant medications: Mycophenolate, azathioprine, and cyclophosphamide are examples of medications that prevent your immune system from attacking healthy tissue. They could have negative side effects as well, such an elevated risk of infection.
- Other medications: In the event that first-line medications aren’t working for you, your heathcare provider can recommend rituximab, or intravenous immunoglobulin as an alternative.
- Rituximab: A monoclonal antibody directed against B cells that cause issues.
- Intravenous immunoglobulin: In order to assist lower the antibodies that lead to your diagnosis, healthy antibodies—proteins produced by your immune system to combat foreign substances—will be injected into your vein through a needle.
- Antibiotic: Infections can occur in pemphigus blisters under certain situations. Your healthcare provider could recommend antibiotics to treat the illness if this occurs.
To verify the effectiveness of treatment, your healthcare provider will conduct regular blood and/or urine tests and monitor your condition routinely. These tests also assess any adverse effects of medications used in pemphigus treatment.
While treatment proves successful for many, full recovery may take years. Some individuals may need ongoing maintenance medication to prevent the return of symptoms. Hospitalization may be necessary for those with severe wounds or infections requiring medical care.
