ERYTHEMA MULTIFORME

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INTRODUCTION

Erythema – Redness ; Multiforme – Polymorphic

First Classified by Bastuji – Garin et al to separate from SJS / TEN in 1922

described by Hebra in 1866 as Erythema exudativum Multiforme

DEFINITION

Acute, Recurrent, self-limiting Cutaneous and/or Mucocutaneous eruptions characterised by target –shaped plaques commonly over extremities and face.

Assoc with HSV / Mycoplasma pneumonia infections

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EPIDEMIOLOGY

Male : Female = 3:2

Occurs in the young / adoloscents

Recurrent in 30 %

Familial clustering s/o Infective Etiology

Assoc with HLA – DQB1 * 0301 Allele

AETIOLOGY

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CLASSIFICATION

EM Minor : Cutaneous without Mucous inv

EM Major (EMM) : Cutaneous + Mucous inv

Mucosal EM : Fuch’s Ectodermosis Pluriorificialis

HAEM

MPAEM

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CLINICAL FEATURES

Prodromal Features

Prev Attack (30%)

Preceding Infection

Fever (T>103.1 deg C)

Cutaneous Lesions

Mucous Lesions

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CUTANEOUS LESIONS

Symmetrical, Acral, Centripetal Rash

Extensor aspect : Extremities > Face > Neck > Trunk

Predilection to Sun-Exposed Areas / Koebnerization

Well-defined, circular, blanching papule / plaque that persists for 01 week – Self-limiting in 04 weeks – Persistent / Continuous EM

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CUTANEOUS LESIONS

Typical Target Lesion :-

1. Dusky Central Disk / Bulla

(later Violaceous / Purpuric)

2. Infiltrated Pale Ring

3. Erythematous edematous halo

Larger lesions show Central

Bulla and Marginal ring of vesicles -

- Herpes Iris of Bateman

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TARGET LESIONS

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CUTANEOUS LESIONS

Raised Atypical Target lesions / Targetoid lesions

SJS-TEN lesions – mostly Macular

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MUCOSAL LESIONS

70% Cases

Lips – Cutaneous

Target Lesions

Serous Crusting

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MUCOSAL LESIONS

LIPS – Mucosal (Erosions / Vesicles / Bullae)GINGIVAL / Ventral TongueHard Palate – SparedPharynx / Larynx / Trachea / BronchiConjunctivitis B/L with Vesicles / ErosionsNasal / Urethral / Anal Mucosae

2/3 Mucosal Sites sans Cutaneous inv

– FUCH’s SYNDROME

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aa

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DDx

ss

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TREATMENT

Treating underlying Etiology

Reducing morbidity – Oral CS

Anti-Virals ?

Macrolides / Quinolones for MPAEM

Oral EM – Topical CS / Anesthetics / Antacids

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RECURRENT EM

Anti-Virals

Topical Acyclovir ?

Azathioprine

Thalidomide

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THANK YOU