Erythema Multiforme

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Contents 1 Introduction 2 Etiology 3 Epidemiology 4 Pathology 5 Clinical-manifestations 6 Complications 7 Differential-diagnosis 8 Management 9 More General Terms 10 Additional Terms 11 References

Introduction

• Self-limited mucocutaneous reaction.

Etiology

• systemic infection

• Herpes simplex (especially recurrent erythema multiforme minor)
• Mycoplasma pneumoniae
• Yersinia enterocolitica

• sensitivity to pharmacologic agent

• barbiturates
• chlorpropamide
• codeine
• penicillins
• phenylbutazone
• phenytoin
• salicylates
• sulfonamides
• sulfones
• tetracyclines
• thiazides
• allopurinol

• pregnancy
• food allergy
• X-rays
• cancer
• connective tissue disease
• inflammatory bowel disease

Epidemiology

• adolescents & young adults
• more frequent in males than females

Pathology

• dermal blood vessel involvement
• extravasation of erythrocytes with resultant purpura
• perivascular mononuclear infiltrate
• edema of the upper dermis
• eosinophilic necrosis of keratinocytes with subepidermal bulla formation
• toxic epidermal necrolysis ( TEN) in severe cases

Clinical-manifestations

• mucous membrane manifestations

• mucosal erosion
• painful
• lips, oropharynx, nasal, conjunctival, vulvar, anal
• sudden appearance
• pharyngitis
• vulvitis & balanitis may interfere with micturition
• cheilitis & stomatitis may interfere with eating

• skin manifestations

• erythematous plaques on skin &/or mucous membranes
• sudden appearance
• sharply demarcated
• symmetric distribution
• "target" lesions resulting from papule or vesicle formation in the center of a plaque
• resolution without scarring
• lesions may last up to 2 weeks
• predilection for dorsum of hands, palms & soles, feet, face, elbows, knees, penis (50%) & vulva
• pruritis
• positive Nikolsky sign in more severe forms

• other manifestations

• systemic symptoms in more severe forms

• mild fever
• arthralgias
• myalgias
• malaise

• eye manifestations

• anterior uveitis
• corneal ulcers
• conjunctivitis

• often recurrent
• severe life-threatening variant

• necrotizing tracheobronchitis
• meningitis
• renal tubular necrosis
• Stevens-Johnson syndrome -> toxic epidermal necrolysis

Complications

• secondary bacterial infection which may result in scarring especially on mucous membranes of eyes & urethra

Differential-diagnosis

• acute erythematous plaques

• drug eruption
• psoriasis
• secondary syphilis
• urticaria

• acute oral erosions

• Herpes
• pemphigus
• lupus erythematosus

Management

• supportive care
• acyclovir for control of Herpes simplex

• does not shorten duration of symptoms
• chronic suppressive therapy may diminish recurrence

• prevention of secondary bacterial infection
• prednisone 50-80 mg PO QD in divided doses, rapidly tapered

• no controlled studies
• reserve for severely ill patients
• not effective; do not use [5]

More General Terms

• vesiculobullous dermatitis

Additional Terms

• acyclovir (ACV, Zovirax)
• erythema nodosum (septal panniculitis)
• Herpes simplex (HSV) or Herpes hominis
• Nikolsky's sign
• pemphigus
• prednisone (Deltasone, Orasone, Liquid Pred, Meticortin)
• psoriasis
• Stevens-Johnson syndrome (SJS)
• syphilis
• systemic lupus erythematosus
• toxic (bullous) epidermal necrolysis (Lyell syndrome, TEN)
• urticaria (hives)

References

1. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
2. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 883
3. Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 332-35
4. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 169
5. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, American College of Physicians, Philadelphia 1998, 2006, 2009

erythema multiforme