Pemphigus and Bulleus Pemphigoid: Rare Autoimmune Diseases with Blistering

Pemphigus and bulleus pemphigoid are rare autoimmune diseases manifested by blistering of the skin and mucous membranes. These conditions can occur suddenly with no apparent cause. In autoimmune diseases, the immune system turns against parts of one's own body. In pemphigus and bulleus pemphigoid, this leads to the formation of antibodies directed against specific components of the skin, causing blistering.

Pemphigus causes blisters in the epidermis, while bullous pemphigoid forms blisters under the epidermis. In general, pemphigus progresses more severely, and without treatment it can be life-threatening, both due to fluid loss resulting from extensive blistering and concomitant infections.

These diseases usually manifest in older age, sometimes in children. Both are treatable, but treatment often takes many years. Fortunately, they are not contagious, not hereditary and usually not malignant.

Causes of Pemphigus and Bulleus Pemphigoid

The exact mechanism of origin of these diseases is unknown. It is possible that certain medications, such as antibiotics, blood pressure-lowering drugs and antipsychotics, can trigger blistering. In some cases, an infection is responsible, especially in a specific form of pemphigus in South America. Very rarely, malignant tumors can trigger a form of pemphigus (para neoplastic pemphigus).

Symptoms

Pemphigus has several forms, of which pemphigus vulgaris and pemphigus foliaceus are the most common. Pemphigus vulgaris usually first causes blisters or superficial wounds in the mouth, later also on the body. Pemphigus foliaceus results only in blisters, erosions and scabs on the skin, especially on the chest, back and face.

Bulleus pemphigoid results in blistering all over the body on a red surface, often accompanied by intense itching. Oral mucosal erosions or blisters are less prominent here.

Another variant, mucosal pemphigoid or cicatricial pemphigoid, primarily affects the mucous membranes of eyes, mouth and genitals. This form is more difficult to treat and can cause scarring, with possible complications such as blindness or strictures of the esophagus and genitals.

Diagnosis and Treatment

The diagnosis is confirmed by microscopic examination of a skin biopsy and blood tests. Although there is no cure, treatment focuses on suppressing skin and mucosal abnormalities. Medications that suppress the immune system, such as corticosteroids and immunosuppressants, are often prescribed. In bulbar pemphigoid, certain antibiotics may be effective.

Pemphigus and bullous pemphigoid are chronic, with a variable course that often lasts for years. The outlook is generally more favorable if the disease develops in childhood. Self-care includes leaving blisters intact, careful handling of damaged skin, and watching for signs of infection.

Always consult a dermatologist for an accurate diagnosis and treatment plan.