When and how was Stevens-Johnson syndrome (SJS) first described, and what is the background of Stevens-Johnson syndrome?

Updated: Jan 17, 2019
  • Author: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Answer

The syndrome was first described in 1922, when the American pediatricians Albert Mason Stevens and Frank Chambliss Johnson reported the cases of 2 boys aged 7 and 8 years with "an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis." Both cases had been misdiagnosed by primary care physicians as hemorrhagic measles.

Erythema multiforme (EM), originally described by Ferdinand von Hebra in 1866, was part of the differential diagnosis in both cases but was excluded because of the "character of skin lesions, the lack of subjective symptoms, the prolonged high fever, and the terminal heavy crusting." Despite the presence of leukopenia in both cases, Stevens and Johnson in their initial report suspected an infectious disease of unknown etiology as the cause.

In 1950, Thomas divided EM into 2 categories: erythema multiforme minor (more common and as previously described by von Hebra) and erythema multiforme major (EMM). Since 1983, erythema multiforme major and Stevens-Johnson syndrome had been considered synonymous.

In the 1990s, however, Bastuji and Roujeau each proposed that erythema multiforme major and Stevens-Johnson syndrome are 2 distinct disorders. [2] They suggested that the denomination of erythema multiforme should be restricted to patients with typical targets or raised edematous papules, with or without mucosal involvement. This clinical picture is in accordance with the original description by von Hebra.

Bastuji and Roujeau further proposed that the denomination of Stevens-Johnson syndrome should be used for a syndrome characterized by mucous membrane erosions and widespread small blisters that arise on erythematous or purpuric maculae that are different from classic targets.

According to this clinical classification, erythema multiforme major and Stevens-Johnson syndrome could be 2 distinct disorders with similar mucosal erosions, but different patterns of cutaneous lesions. This hypothesis is supported further by a strong correlation between clinical classification and the probable cause.

Conversely, several investigators propose that Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN) represent the same disease at different levels of severity. A unifying classification of "acute disseminated epidermal necrosis" or "exanthematic necrolysis" has been suggested.


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