What is the difference between erythema multiforme major, Stevens-Johnson syndrome, and toxic epidermal necrolysis? This is a critical and important question that is difficult to answer because this nosological nightmare continues to be controversial. The short version of these distinctions is as follows: - Erythema multiforme major is best defined as presentation with targetoid skin lesions that are typical of erythema multiforme, with a more severe variant that is more likely to demonstrate oral lesions, fever, and systemic symptoms. While this variant can be drug-induced, it is more commonly induced by infections such as herpes simplex and Mycoplasma. Some dermatologists consider this to be in the spectrum of Stevens-Johnson syndrome. Microscopically, the keratinocytes are being damaged by lymphocytes (satellite cell necrosis).
- Stevens-Johnson syndrome is most commonly defined as presentation with widespread targetoid lesions that are a flat and atypical when compared to the more defined lesions of erythema multiforme. Lesions are also more frequently purpuric. As in the case of erythema multiforme major, the patients may have fever and systemic symptoms. In contrast to erythema multiforme major, the lesions are more likely to become confluent and develop large areas of blisters and detachment of the epidermis. While some cases are idiopathic or induced by infections, the majority are drug induced. Histologically, the findings are identical to erythema multiforme in that the keratinocytes demonstrates satellite cell necrosis. Some dermatologists arbitrarily define this condition as affecting less than 30% of the body surface, and some authorities even recognize a Stevens-Johnson syndrome/toxic epidermal necrosis overlap syndrome.
- Toxic epidermal necrosis is best defined as a blistering disorder with extensive detachment of the skin that is almost always drug induced, although there are exceptions. Many of the drugs that produce classic Stevens-Johnson syndrome also produced toxic epidermal necrolysis. Targetoid lesions are not usually present but if present are atypical. Microscopically, biopsies are cell poor and cells usually appear to become necrotic without evidence of satellite cell necrosis, suggesting a soluble factor. Some dermatologists arbitrarily differentiate this from Stevens-Johnson syndrome if more than 30% of the cutaneous surface is involved, although many dermatologists feel that toxic epidermal necrolysis and Stevens-Johnson syndrome represent a spectrum of disease.
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