Pemphigus vulgaris is a rare autoimmune skin disease. An immunosuppressive treatment has recently revolutionized its treatment.
What is pemphigus vulgaris?
Pemphigus vulgaris is a form of pemphigus, bullous diseases of the skin and mucous membranes. There are mainly two forms (a third form is much rarer). Pemphigus vulgaris or deep is the most common form that affects 70% of patients. “It causes erosive plaques such as burns of the mucous membranes and skin” describes Pr Pascal Joly, dermatologist at the University Hospital of Rouen, Director of the French reference center for bullous autoimmune diseases. Superficial pemphigus represents 30% of pemphigus cases and is characterized by skin lesions uniquely. Pemphigus vulgaris is a rare disease: per year, 1 new case per 1 million inhabitants. “Before the first treatments appeared in the 1950s, it was a fatal disease.“says Professor Joly.
What causes pemphigus vulgaris?
Pemphigus are autoimmune diseases. That is to say, they are linked to a immune system disorder : instead of recognizing and attacking elements foreign to our body (such as viruses), the immune system recognizes its own constituents and attacks them. “In the case of pemphigus, the immune system produces antibodies directed against interkeratinocyte adhesion proteinsdesmogleins 1 (in superficial pemphigus) and/or 1 and 3 (in pemphigus vulgaris) These proteins have the role of making the keratinocytes adhere to each other“explains Professor Pascal Joly. The loss of adhesion between keratinocytes, cells that make up a major part of the superficial layer of the skin, exposes the dermis.
Pemphigus vulgaris is not contagious
What are the symptoms of pemphigus vulgaris?
The disease is manifested by the formation of intradermal bubbles which burst almost immediately after their appearance creating skin and mucous membrane erosions. Cutaneous lesions are found in particular on the face and scalp, on the chest, back, arms and legs. Mucosal lesions occur most often in the mouth and in the esophagus – these lesions being painful and preventing feeding-, more rarely at the genital level.
Is it contagious ?
pemphigus vulgaris is not contagious as it is not an infectious disease but of an autoimmune disease, therefore linked to a dysregulation of the immune system.
“The diagnosis is guided by clinical lesions and confirmed by a skin biopsy highlighting the separation of the cells of the epidermis, and by tests showing the presence of antibodies against desmogleins” informs Professor Pascal Joly. These antibodies can be demonstrated in the skin by direct immunofluorescence or in the blood by immunofluorescence or with the ELISA method. “The level of antibodies in the blood parallels the severity of the disease: the more antibodies there are, the more severe the disease.“says Pr Pascal Joly who explains to us that the blood antibody level detection is also a means of monitoring patients in remission in order to detect a recurrence early (when the level of antibodies rises).
What is the treatment for pemphigus vulgaris?
Pemphigus vulgaris was treated for a long time only with long-term corticosteroids. However, cortisone taken for years has side effects. An immunosuppressant, Rituximab, is now used in addition to corticosteroid therapy. Taken for 3 to 6 months, it destroys B cells that make pemphigus antibodies. “When treatment is stopped, the bone marrow will remake B lymphocytes, which by complex mechanisms are no longer self-reactive in most patients” informs Professor Pascal Joly. “Using Rituximab makes it possible to quickly reduce corticosteroids in 3 months in people who have a mild form of pemphigus vulgaris, in 6 months in those with a severe form” describes Pr Joly. The rate of complete remission weaned from cortisone in patients receiving Rituximab is 90% versus 30% for those receiving only cortisone. Pr Joly announces that a new study is in progress at the Rouen University Hospital to see if this treatment could be given preventively to prevent a relapse of the disease (when the level of antibodies rises in the blood).
Thanks to Pr Pascal Joly, dermatologist at the University Hospital of Rouen, Director of the French reference center for bullous autoimmune diseases.