Dermatology - Pemphigus Vulgaris

Dermatology - Pemphigus Vulgaris
This is a bullous autoimmune illness characterized by acantholysis, which can be either acute or chronic. The prevalence of the condition is higher among individuals of Jewish and Mediterranean ancestry, with the typical age of onset being between 40 and 60 years.


Pemphigus vulgaris typically initiates in the oral mucosa, and a considerable amount of time may pass before lesions manifest. Excruciating and sensitive mouth sores can hinder sufficient consumption of food.
Epistaxis, hoarseness, dysphagia, weakness, malaise, and weight loss are some symptoms that may manifest.

Round or oval fluid-filled sacs and blisters with a watery substance, soft and easily burst, appear on healthy skin, and can be randomly spread out and separate, confined to a specific area or spread throughout with an irregular arrangement. The scalp experiences significant erosion, which results in easy bleeding and the formation of crust. Due to the high susceptibility of blisters to rupture, a significant number of patients exhibit exclusively painful erosions.
The application of lateral finger pressure near lesions causes the displacement of intact epidermis, resulting in erosion, which is known as the Nikolsky sign. Applying pressure on the bulla causes the blister to extend laterally.

Diagnosing the condition becomes challenging when just oral lesions are observed. In such cases, confirming the clinical suspicion requires conducting a sample of both the skin and mucous membrane, followed by immunohistology analysis. The differential diagnosis include all acquired bullous diseases.

Administer glucocorticoids (at a dosage of 2 to 3 mg/kg prednisone) until the development of new blisters stops and the Nikolsky sign disappears. Subsequently, there will be a fast decrease to half of the original dosage until resolution, followed by a gradual decrease to the lowest effective dose for maintenance. Immunosuppressive drugs are administered concurrently to achieve their glucocorticoid-sparing action. Rituximab is administered in severe situations.

​Plasmapheresis may be beneficial in people with uncontrolled illness by lowering antibody levels. Gold treatment is effective for less severe instances. Supportive care encompasses several treatments such as cleansing baths, wet dressings, the application of glucocorticoids on the skin and directly into the affected area, as well as the use of antimicrobials to treat any subsequent infection. Rectify hydration and electrolyte imbalance and oversee the amelioration of cutaneous lesions and adverse effects associated with medication.