Erythema multiforme: a practical approach to recent advances.

Abstract

In this edition of Pediatric Dermatology Samimi and Siegfried report the interesting and provocative case of a 9-year-oldgirlwithhypocomplementemicsystemiclupus erythematosus and severe nephritis. Over an approximate 3-week time span this very ill patient was treated withsevendiAerentagentsincludingintravenousandoral corticosteroids.Within2‐3weeks(andwhileonsteroids) she developed a fever and a widespread eruption involving her face, trunk, and the mucous membranes of her eyes,mouth,andvulva.Intravenouscefuroximewasthen added.Atthattimeadermatologyconsultantsuggesteda diagnosis of bullous lupus erythematosus or Stevens‐ Johnson syndrome (SJS). The skin biopsy favored SJS and a direct immunofluorescence study was negative. Furosemide was discontinued as ‘‘the most likely cause’’ but the disease progressed over the next 2 days. At this pointa4-daycourseofintravenousimmunoglobin(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 4daysafterstartingIVIgandthepatientwasdischarged3 weeks later. There are several important new points regarding severe erythema multiforme (EM) that are illustrated by thiscasereportandotherrecentpublications.Thisarticle 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).