Pemphigus — Clinical Case (Wikimedia Commons)

Diagnosis: Pemphigus

Watercolour drawing of feet and lower legs affected with acute pemphigus. Keywords: Godart, Thomas. Clinical photograph sourced from Wikimedia Commons (CC BY 4.0). Attribution: Godart, Thomas.

Clinical Presentation

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.

Clinical History

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.

Treatment

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.

Differential Diagnosis

Bullous pemphigoid, Paraneoplastic pemphigus, IgA pemphigus, Hailey-Hailey disease, Erythema multiforme, HSV

Key Learnings

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