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11 maj 2017 · Histological analysis shows that pemphigus vulgaris blisters develop deep in the epidermis or oral epithelium above the basal layer, whereas pemphigus foliaceus blisters occur in the...
Fig 1 Diagnosis of pemphigus. Diagnosis requires clinical presentation and histopathology that are consistent with pemphigus and either a positive direct immunofluorescence (DIF) microscopy result or serologic detection of autoantibodies against epithelial cell surface antigens.
In general, adults with pemphigus and high TPMT activity are treated with normal doses of azathioprine (≥2.5 mg/kg/d). Patients with intermediate or low TPMT activity should receive a lower maintenance dose (≥0.5 to 1.5 mg/kg/d) depending on level of enzyme activity.
Key words: CD20 inhibitor; consensus; guidelines; pemphigus foliaceus; pemphigus vulgaris; treatment. Pemphigus encompasses a spectrum of rare mucocu-taneous bullous diseases that are autoimmune in origin. Because of the rarity of these diseases, it can take patients months before their pemphigus is diagnosed, during which time many are treated ...
We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.
15 maj 2020 · For initial treatment of moderate-to-severe pemphigus vulgaris/foliaceus, recommended treatment options now include rituximab (anti-CD20 antibody) in combination with corticosteroids (in the 2015 version, this immunosuppressant was recommended only for treatment-resistant disease).
Our improved understanding of the autoantibody-driven effector phase of pemphigus has led to the introduction of novel therapies that target pathogenic autoantibodies such as immunoadsorption and drugs that block pathogenic autoantibody-induced cell signalling events.
