CURRENT Essentials of Critical Care.pdf

Chapter 18 Dermatology 269Pemphigus Vulgaris■ Essentials of Diagnosis• Flaccid, easily ruptured blisters on noninflamed skin; after rupture,nonhealing crusted erosions remain; 50% begin withpainful oral erosions• Superficial detachment of skin after pressure or trauma (Nikolskysign)• Skin biopsy: characteristic intraepidermal cleft just above basalcell layer with separation of keratinocytes from one another(acantholysis)• Direct immunofluorescence of normal-appearing skin shows intercellularIgG and complement throughout epithelium• Rare, life-threatening disease (mortality rate 60—90% before;10% after advent of corticosteroids) caused by circulating IgGautoantibodies directed against intercellular substance of epidermis■ Differential Diagnosis• Erythema multiforme, Stevens-Johnson syndrome, toxic epidermalnecrolysis• Bullous drug eruptions• Bullous impetigo• Other primary blistering diseases (bullous pemphigoid, dermatitisherpetiformis)■ Treatment• Discontinue drugs known to be associated with pemphigus(penicillamine, captopril)• Prednisone, 60–120 mg/d, in combination with azathioprine,100–150 mg/d, usually effective• Prior to therapy, patient should be evaluated for contraindicationsto systemic steroids• When control of the blistering is achieved, prednisone is graduallyreduced as tolerated• Methotrexate, cyclophosphamide, mycophenolate mofetil, cyclosporine,gold, plasmapheresis alternative modalities• Whirlpool treatments helpful in removing crusts from lesions• Oral mucosal erosions may benefit from topical steroids, antiseptics,viscous lidocaine, attention to oral hygiene■ PearlParaneoplastic pemphigus is a very rare complication of cancer, mostoften non-Hodgkin’s lymphoma, with overlapping clinical and histologicalfeatures to pemphigus vulgaris.ReferenceFellner MJ, Sapadin AN: Current therapy of pemphigus vulgaris. Mt Sinai JMed 2001;68(4-5):268. [PMID: 11514914]