Erythema Multiforme syndrome


## Basic introduction

  • Erythema multiforme (EM) is an acute and self-limiting allergic reaction that is triggered mainly by infections (such as herpes simplex virus, HSV) and drugs.
  • EM exists in "multiple forms" with a wide variety in its clinical presentation.
  • EM is subdivided into two main classes: Erythema multiforme minor (EM minor) and Stevens-Johnson syndrome (EM major or SJS)
  • The presentations of the two classes of EM overlap to a small extent
  • EM minor (constituting 80% of EM) is the less severe of the two types

## Pathogenesis

  • There is infiltration of mononuclear cells into the dermoepidermial junction
  • This leads to the formation of vesicles acrally on fingers, palms, soles, and nail beds

## Etiology of EM

  • 50% of all cases are idiopathic
  • HSV (majority of known cases)
  • Other viral infections such as HIV, Herpes zoster, HCV, and CMV
  • Fungal infections
  • Bacterial infections (like streptococcal and mycoplasma infections)
  • Physical factors such as radiotherapy, cold, and sunlight
  • Cancers

## Statistics

  • 50% of cases of EM < 20 years old
  • Males are > females
  • 1/3 of EM cases will recur
  • The fatality of EM major is 5 -15%

## EM minor

  • Raised, edematous papules distributed on fingers, palms, soles, and nail beds (acral distributions)

## EM major

  • Raised, edematous papules distributed on fingers, palms, soles, and nail beds (acral distributions)
  • Involvement of one or more mucous membranes
  • Epidermal detachment (in < 10% of total body surface area but it can be >30% in case of toxic epidermal necrolysis, TEN)
  • Blisters may spread on the trunk and face
  • Erythematous or pruritic macules
  • Mucous membrane erosions
  • Fever
  • Prostration
  • Stomatitis and/or cheilitis (inflammation of the lips) that lead to poor feeding
  • Vulvitis
  • Balanitis
  • Conjunctivitis that may end up leading to keratitis
  • Generalized lymphadenopathy
  • Clinical review
  • Investigations such as CXR, culture & sensitivity and skin biopsy to establish the underlying cause(s)
  • Contact dermatitis
  • Drug eruptions
  • Pemphigoid
  • Pemphigus
  • Pityriasis rosea
  • Urticarial vasculitis
  • Avoiding any known underlying cause
  1. American Osteopathic College of Dermatology, Erythema multiforme. Accessed on 11 January 2017
  2. Drug allergy: diagnosis and management of drug allergy in adults children and young people; NICE Clinical guideline (September 2014)
  3. Kenyan Ministry of Health. Clinical guidelines for management and referral of common conditions at levels 4-6. Hospitals. 2009; 3:259-261.
  4. Ministry of Health, Kenya. Kenya Essential Medicine List (2016).
  5. Woo SB, Challacombe SJ; Management of recurrent oral herpes simplex infections. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Mar;103 Suppl:S12.e1-18.
  • Identification and removal of the trigger factor(s) where possible
  • EM minor cases often resolve by themselves within 2-4 weeks (they may not require treatment)
  • Early treatment of HSV with acyclovir
  • If EM keeps on recurring, administer low-dose of acyclovir continuously
  • For SJS (EM major) transfer to the burn unit and administer: cyclophosphamide, pooled gamma globulin, oral cyclosporine
  • Corticosteroid therapy (such as prednisolon  tablets) in SJS (this is controversial and discouraged patients with severe mouth and throat sores as patient easily give in to fatal respiratory infections)
  • Clean the skin with normal saline
  • In case there is suspected bacterial eye infection(s), apply tetracycline 1% ointment
  • IV fluids to patients who are unable to feed orally
  • Cradle nursing in severe cases
  • Keep the patient warm
  • Use antiseptic mouth wash in case of stomatitis
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