Erythema and desquamation

ERYTHEMA MULTIFORME

L51.9

DESCRIPTION

An acute, self-limiting and commonly recurrent inflammatory eruption of the skin with variable involvement of the mucous membranes and without systemic symptoms.

Symmetrically distributed crops of target lesions (dark centre, an inner, pale ring surrounded by an outer red ring) often involving palms and soles are characteristic. This condition is mainly caused by:

  • medicines, e.g. sulphonamides, phenytoin, phenobarbitone,
  • exposure to toxic substances, and
  • infections, e.g. herpes simplex and mycoplasma.

Complications include:

  • conjunctivitis,
  • uveitis,
  • corneal scarring,
  • fluid loss,
  • infections,
  • anaemia, and
  • oesophageal strictures.

DIAGNOSTIC CRITERIA

Iris or target lesions consisting of a dark centre, an inner pale ring and an erythematous outer border. In erythema multiforme these lesions are pathognomonic.

Erythematous macules evolve into papules, vesicles, bullae, urticarial plaques or patches of confluent erythema. The centre of the lesion may be vesicular, purpuric or necrotic.

Erythema multiforme minor

Prodromal symptoms are generally absent. Symmetric crops of skin lesions of diverse morphology, primarily on the extensor surfaces of the arms and legs and often including soles and palms with relative sparing of the mucous membranes and the trunk.

Erythema multiforme major (often equated with Stevens-Johnson syndrome)
A serious, systemic condition involving the skin and at least two mucous membranes.
Eruption may be preceded by non-specific prodromal symptoms including:

  • malaise,
  • fever,
  • rigors, or
  • upper respiratory tract infection.

Cutaneous lesions tend to rupture, leaving the skin denuded leading to fluid loss, with high risk of infection. Anaemia is common. The oral mucosa is frequently involved.

GENERAL AND SUPPORTIVE MEASURES

  • May require care in high or intensive care unit.
  • Examine daily for systemic involvement, infection and ocular lesions. If infection is suspected, send blood and skin lesion specimens for culture and sensitivity before initiating antibiotic therapy.
  • Do not puncture bullae or vesicles.
  • Cool compresses and wet dressings.
  • Encourage oral fluids, to prevent adhesions.
  • Regular supervised oral, genital and eye care to prevent adhesions and scarring.
  • Maintain fluid balance. Beware of shock.
  • Nasogastric feeds if unable to eat, IV alimentation if enteral feeds are not possible.
  • Stop all potentially causative medicines.

MEDICINE TREATMENT

For pain:

These patients require effective pain control.
Change of dressing protocol: See: Pain Control.

Dressings

Skin hygiene, daily cleansing and bland, non-adherent dressings as needed.

Do not use silver sulfadiazine if Stevens-Johnson Syndrome is thought to be due to cotrimoxazole or other sulphonamide

Antibiotic therapy

For secondary infections
Use IV antibiotics if the oral route cannot be used.

  • Cloxacillin, IV, 50 mg/kg/dose 6 hourly.

OR

  • Flucloxacillin, oral, 25mg/kg/dose 6 hourly.

LoEIII [1]
LoEIII [2]

OR (if flucloxacillin is unavailable)

  • Cephalexin, oral, 6.25–12.5 mg/kg/dose 6 hourly.

Reconsider choice of antibiotic when the results of cultures become available or the child does not improve.

If Herpes Simplex Virus (HSV) is suspected to be the cause:

  • Aciclovir, oral, 250mg/m^2/dose 8 hourly for 7 days.

For oral lesions:

  • Chlorhexidine 0.2%, 15 mL as a mouthwash.
    • Use as needed.
    • Do not swallow.

Note:
The use of systemic corticosteroids is not recommended.

REFERRAL

  • Erythema multiforme not responding to adequate therapy.
  • Erythema multiforme with ocular involvement.

STEVENS-JOHNSON SYNDROME (SJS)/TOXIC EPIDERMAL NECROSIS (TEN)

L51.1/ L51.2

DESCRIPTION

Life-threatening, acute hypersensitivity reaction with systemic upset, epidermal necrosis, and mucous membrane involvement. TEN and SJS are different ends of the same spectrum: in TEN epidermal necrosis involves > 30% of body surface area, while in SJS the involvement is < 10%.

This condition is usually due to medication, e.g. sulphonamides, nonnucleoside reverse transcriptase inhibitors (especially nevirapine), antiepileptics (phenytoin, phenobarbitone, carbamazepine, lamotrigine), allopurinol, laxatives (phenolphtalein).

Complications include:

  • Dehydration, electrolyte disturbances and shock,
  • Hypoalbuminaemia,
  • hypo- and more commonly hyperthermia,
  • high output cardiac failure,
  • secondary infection and sepsis; and
  • adhesions and scarring.

DIAGNOSTIC CRITERIA

Cutaneous lesions may start as a dusky red macular rash, progressing to confluence with epidermal necrosis and large flaccid blisters which rupture, leaving large areas of denuded skin. Mucous membrane erosions are common and multi- organ involvement may be present.

GENERAL AND SUPPORTIVE MEASURES

  • May require care in high or intensive care unit.
  • Examine daily for systemic involvement, infection and ocular lesions. If infection is suspected, send blood and skin lesion specimens for culture and sensitivity before initiating antibiotic therapy.
  • Do not puncture bullae or vesicles.
  • Cool compresses and wet dressings.
  • Regular supervised oral, genital and eye care to prevent adhesions and scarring.
  • Encourage oral fluids, to prevent adhesions.
  • Maintain fluid balance. Beware of shock.
  • Nasogastric feeds if unable to eat, IV alimentation if enteral feeds are not possible.
  • Stop all potentially causative medicines.

MEDICINE TREATMENT

For pain:

These patients require effective pain control.
Change of dressing protocol: See: Pain Control.

Dressings

Skin hygiene, daily cleansing and bland, non-adherent dressings as needed.

Do not use silver sulfadiazine if Stevens-Johnson Syndrome is thought to be due to cotrimoxazole or other sulphonamide.

Empiric antibiotic therapy

For secondary infections
Use IV antibiotics if the oral route cannot be used.

  • Cloxacillin, IV, 50 mg/kg/dose 6 hourly.

OR

  • Flucloxacillin, oral, 25mg/kg/dose 6 hourly.

LoEIII [1]
LoEIII [3]

OR (if flucloxacillin is unavailable)

  • Cephalexin, oral, 6.25–12.5 mg/kg/dose 6 hourly.

Reconsider choice of antibiotic when the results of cultures become available or the child does not improve.

For oral lesions:

  • Chlorhexidine 0.2%, 15 mL as a mouthwash.
    • Use as needed.
    • Do not swallow.

Note:
The use of systemic corticosteroids is not recommended.

REFERRAL

  • Discuss with a specialist, if considering re-initiation of medicine treatment.