Erythema multiforme: a practical approach to recent advances.

In this edition of Pediatric Dermatology Samimi and Siegfried report the interesting and provocative case of a 9-year-oldgirlwithhypocomplementemic systemic lupus erythematosus and severe nephritis. Over an approximate 3-week time span this very ill patient was treated withsevendi€erentagents including intravenousandoral corticosteroids.Within 2±3weeks (andwhile on steroids) she developed a fever and a widespread eruption involving her face, trunk, and the mucous membranes of her eyes,mouth,andvulva. Intravenouscefuroximewas then added.At that timeadermatologyconsultant suggesteda diagnosis of bullous lupus erythematosus or Stevens± Johnson syndrome (SJS). The skin biopsy favored SJS and a direct immuno ̄uorescence study was negative. Furosemide was discontinued as ``the most likely cause'' but the disease progressed over the next 2 days. At this pointa4-daycourseof intravenous immunoglobin(IVIg) (750 mg/kg/day) and the reinstitution of intravenous methylprednisolone 1 mg/kg every 12 hours was begun. Hydroxychloroquine and cefuroxime were discontinued and replaced by vancomycin and cefazidime. Her condition continued to deteriorate for the next 3 days with critical care management including skin debridement, transfusion of blood and albumin, and intubation with mechanical ventilation. Gradual improvement began 4daysafter startingIVIgandthepatientwasdischarged3 weeks later. There are several important new points regarding severe erythema multiforme (EM) that are illustrated by this case report andother recent publications.This article will comment on the practical importance for the involved clinician of increased susceptibility to EM in high-risk patient groups (disease predisposition), genetic predisposition, and the role of drugs in causing and treating SJS/toxic epidermal necrolysis (TEN).