Diagnosis: Pemphigus
Watercolour drawing of the right axilla of man illustrating the eruption seen in pemphigus vegetans. This drawing shows the older lesions present when the patient was admitted to the Hospital. They il. Clinical photograph sourced from Wikimedia Commons (CC BY 4.0). Attribution: Yetts, Walter Perceval.
PV: flaccid bullae that easily rupture leaving painful erosions. Mucosal involvement in >50%. Positive Nikolsky sign (lateral pressure causes epidermal separation). PF: superficial erosions with crusting, primarily on trunk/face, no mucosal involvement.
Peak onset 40-60 years. May be drug-induced (ACE inhibitors, penicillamine, rifampin). Sephardic Jews and Mediterranean populations at higher genetic risk. Progressive course without treatment — historically fatal.
First-line: systemic corticosteroids + rituximab (now first-line per guidelines). Steroid-sparing: mycophenolate, azathioprine. IVIG for acute flares. Monitor: anti-desmoglein titers correlate with disease activity.
Bullous pemphigoid, Paraneoplastic pemphigus, IgA pemphigus, Hailey-Hailey disease, Erythema multiforme, HSV
Rituximab has changed the treatment paradigm — now recommended as first-line with corticosteroids. The desmoglein compensation theory explains mucosal vs cutaneous involvement. Anti-desmoglein ELISA is useful for diagnosis and monitoring disease activity.
Tags: pemphigus, autoimmune, blistering, desmosome