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Erythema Multiforme, Reactive Infectious Mucocutaneous Eruption, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis


Erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) are acute hypersensitivity reactions characterized by cutaneous and mucosal necrosis. These syndromes represent a hypersensitivity reaction to a precipitating cause, usually infectious organisms or drugs. These disorders were historically thought to represent a spectrum of the same disease process, and inconsistent use of these disease names led to further confusion. A consensus definition was published in 1993, and current literature favors that EM is distinct from the SJS/TEN spectrum of disease. The differential diagnosis of vesiculobullous eruptions is listed in Table 195.1 .

Table 195.1
Vesiculobullous Eruptions
Modified from Nopper AJ, Rabinowitz LG. Rashes and skin lesions. In: Kliegman RM, ed. Practical Strategies in Pediatric Diagnosis and Therapy . Philadelphia: WB Saunders; 1996.
ENTITY CLINICAL CLUES
INFECTIOUS
Bacterial
Staphylococcal scalded skin syndrome

  • Generalized, tender erythema

  • Nikolsky sign

  • Occasionally associated with underlying infection such as osteomyelitis, septic arthritis, pneumonia

  • Desquamation and moist erosions observed, especially in intertriginous areas

  • More common in children <5 yr of age

Bullous impetigo Localized blisters from staphylococcal infection
Viral
Herpes simplex

  • Grouped vesicles on erythematous base

  • May be recurrent at same site—lips, eyes, cheeks, hands

  • Reactivated by fever, sunlight, trauma, stress

Varicella

  • Crops of vesicles on erythematous base (“dewdrops on rose petal”)

  • Highly contagious

  • May see multiple stages of lesions simultaneously

  • Associated with fever

Herpes zoster

  • Grouped vesicles on erythematous base limited to one or several adjacent dermatomes

  • Thoracic dermatomes most commonly involved in children

  • May be varicella vaccine associated

  • Usually unilateral

  • Burning, pruritus

Hand-foot-mouth syndrome (coxsackievirus infection) Prodrome of fever, anorexia, sore throat Oval blisters in acral distribution, usually few in number Shallow, oral erosions on erythematous base in oral mucosa Highly infectious Peak incidence in late summer and in fall
HYPERSENSITIVITY
Erythema multiforme

  • Target lesions on acral sites

  • May have involvement of mucosal surfaces

  • Associated with herpes simplex virus infection

Reactive infectious mucocutaneous eruption

  • Prodrome of fever and cough

  • Severe mucositis of 2+ mucosal surfaces

  • Scarce/absent rash

  • Associated with Mycoplasma pneumonia , Chlamydophila pneumonia , or viral respiratory infection

Stevens-Johnson syndrome and toxic epidermal necrolysis

  • Prodrome of fever, headache, malaise, sore throat, cough, vomiting, diarrhea

  • Extensive epidermal necrosis with mucosal involvement

  • Frequently related to drugs (e.g., sulfonamides, anticonvulsants)

  • Nikolsky sign

EXTRINSIC
Contact dermatitis

  • Irritant or allergic

  • Distribution dependent on the irritant or allergen

  • Distribution helpful in identifying cause

Insect bites

  • Occur occasionally following flea or mosquito bites

  • May be hemorrhagic bullae

  • Often in linear or irregular clusters

  • Very pruritic

Burns

  • Irregular shapes and configurations

  • May be suggestive of abuse

  • Bullae with second- and third-degree injuries

Friction

  • Usually on acral surfaces

  • May be related to footwear

  • Often activity-related

MISCELLANEOUS
Urticaria pigmentosa

  • Red-brown macules and papules; bullous lesions are rare

  • Darier sign

  • Lesions appear in infancy and spontaneously resolve in childhood

Miliaria crystallina

  • Clean, 1- to 2-mm superficial vesicles occurring in crops

  • Rupture spontaneously

  • Intertriginous areas, especially neck and axillae

Hereditary: epidermolysis bullosa, incontinentia pigmenti, epidermolytic hyperkeratosis
Autoimmune: linear immunoglobulin A disease, bullous pemphigoid, dermatitis herpetiformis

Erythema Multiforme

EM is a common, self-limiting, acute hypersensitivity syndrome characterized by the abrupt onset of round, deep red, well-demarcated macules and papules with a dusky gray or bullous center. The size may vary, but most lesions are approximately 1 cm in diameter. The classic target lesion consists of three concentric rings: The outermost is red, the intermediate is white, and the center is a dusky red or purple. These can progress to edematous plaques or bullae. If blistering occurs, it is circumscribed and involves less than 10% of the body surface area. Cutaneous lesions are symmetrically distributed and commonly involve acral areas such as the hands, feet, elbows, and knees. Involvement of the ocular, oral, and genital mucosa can be seen in some cases.

Most EM cases in children are precipitated by herpes simplex virus infection, although the infection may no longer be apparent by the time EM develops. Symptomatic treatment is usually sufficient. Oral antihistamines help suppress pruritus, stinging, and burning. The use of systemic corticosteroids is controversial but may be considered for severe mucosal disease. Antiviral medications targeting herpes simplex virus do not alter the course of the EM, although children with recurrent EM may be candidates for prophylactic antivirals. The prognosis is excellent, with most lesions lasting no more than 2 weeks. Healing occurs without scarring.

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