Epidermal Necrolysis Disease Spectrum

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INTRODUCTION Erythema multiforme is an acute mucocutaneous hypersensitivity reaction. Although once believed to be distinct diseases, many observers currently consider erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis (TENS) to represent a mild to severe continuum of the same process. Erythema multiforme minor represents the mildest form. These three entities share certain clinical, histologic, and etiologic characteristics in common. Although ocular involvement in erythema multiforme minor is rare, it is seen frequently in both Stevens-Johnson syndrome and TENS. The majority of cases occur in children and young adults. Most cases follow exposure to a drug or infectious agent. The most frequently implicated organisms have been herpes simplex, Mycoplasma pneumoniae, and Streptococcus. Drugs include antibiotics and seizure medications. Recurrences can occur and the disease is fatal in 10% of cases.

CLINICAL PRESENTATION Erythema multiforme minor is characterized by round erythematous rapidly progressive mucocutaneous macules or papules. The borders are bright red with central petichiae, vesicles, or purpura. Conjunctivitis with blisters and ulcerations can be seen, and secondary infection is common. Lesions may coalesce and become generalized. Burning may be significant, but pruritis is generally absent. These lesions usually resolve over one to several weeks, but postinflammatory hyper- or hypopigmentation may occur. In EM major (Stevens-Johnson syndrome) prodromal symptoms occur in 50% of cases and include fever, malaise, sore throat, arthral-gia, vomiting, and diarrhea. Mucocutaneous involvement shows bullous lesions which become hemorrhagic and necrotic, leading to extensive denuded areas of skin and mucous membrane including the mouth and conjunctiva. Scarring results in lagophthalmos, trichiasis, symblepharon,

Erythema Multiforme/Stevens-Johnson Syndrome (Contd.)

and eyelid malpositions such as entropion and ectropion. Loss of mucin producing goblet cells contributes to a severe dry eye syndrome. TENS produces even more severe disease characterized by large flaccid bullae that quickly progress to peeling off of epithelium in great sheets exposing the raw, weeping dermis. This is a medical emergency and may be life-threatening.

Early Photos Stevens Johnson Syndrome
(Courtesy of Robert A. Goldberg, M.D.) (Courtesy of Charles S. Soparkar, M.D.)

HISTOPATHOLOGY In early lesions, there are vacuolated basal epidermal cells, mild spongiosis, lymphocytes along the epidermal-dermal junction and migrating into the epidermis ("exocytosis"), and a mild perivascular lymphohistiocytic infiltrate in the superficial dermis. Apoptosis of individual basal cells results in eosinophilic, rounded keratinocytes that may be anucleate or have pyknotic nuclei; these are the hallmark of erythema multiforme. Lymphocytes may surround dying keratinocytes ("satellite cell necrosis"). More edematous lesions have greater spongiosis and papillary edema, and a more intense inflammatory infiltrate.

Satellite Cell Necrosis

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes cicatricial ocular pemphigoid, IgA linear dystrophy, rosacea, and pityriasis rosea.

TREATMENT Clinical diagnosis may be difficult; therefore a skin biopsy can be extremely helpful. Withdrawal of the causative agent is essential. For EM minor symptomatic treatment is offered. Acute episodes should be managed with topical corticosteroids and ocular lubrication. For recurrent

Erythema Multiforme/Stevens-Johnson Syndrome (Contd.)

disease, prophylactic oral acyclovir may be effective in reducing episodes. For EM major hospitalization is required. Systemic immunosuppressive agents such as cyclophosphamide or azathioprine, either alone or in combination with systemic steroids are indicated. Local measures such as tear replacement, and topical steroids and antibiotics are used for ocular involvement. Debridement of the fornices with lysis of symblephara using a glass rod may be helpful. Surveillance cultures should be taken every few days, and secondary infections should be treated aggressively. Once the acute phase has resolved surgical therapy including skin grafts to treat lagophthalmos, lysis of symblephara with or without conjunctival or amniotic membrane grafting, and tarsorraphy may be necessary. Trichiasis may require treatment with epilation, electrocautery, or cryosurgery.


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