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W ong A et al.
468 Rev Assoc Med Bras 2016; 62(5):468-473
REVIEW ARTICLE
Stevens-Johnson syndrome and toxic epidermal necrolysis: a review
Anthony Wong1, A ndrey Augusto Malvestiti2, Mariana de Figueiredo Silva Hafner3*
1Professor of Clinical Toxicology, Medical Director of Centro de Assistência Toxicológica (Ceatox), Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (FMUSP), São Paulo, SP, Brazil
2Assistant Physician at the Ceatox, Hospital das Clínicas, FMUSP. Dermatologist at Hospital Israelita Albert Einstein (HIAE), São Paulo, SP, Brazil
3Assistant Physician at Clínica de Dermatologia da Santa Casa de São Paulo. Dermatologist at the HIAE, São Paulo, SP, Brazil
Summary
Study conducted at Centro de Assistência
Toxicológica (Ceatox), Instituto da Criança,
Hospital das Clínicas, Faculdade de
Medicina, Universidade de São Paulo
(FMUSP), São Paulo, SP, Brazil
Article received: 5/6/2015
Accepted for publication: 5/16/2015
*Correspondence:
Address: Av. Dr. Enéas de Carvalho
Aguiar, 647
São Paulo, SP – Brazil
Postal code: 05403-900
Financial support: Sanofi Aventis.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are un-
common, acute and potentially life-threatening adverse cutaneous drug reac-
tions. These pathologies are considered a hypersensitivity reaction and can be
triggered by drugs, infections and malignancies. The drugs most often involved
are allopurinol, some antibiotics, including sulfonamides, anticonvulsants such
as carbamazepine, and some non-steroid anti-inflammatory drugs (NSAIDs).
Necrosis of keratinocytes is manifested clinically by epidermal detachment, lead-
ing to scalded skin appearance. The rash begins on the trunk with subsequent
generalization, usually sparing the palmoplantar areas. Macular lesions become
purplish, and epidermal detachment occurs, resulting in flaccid blisters that con-
verge and break, resulting in extensive sloughing of necrotic skin. Nikolsky's
sign is positive in perilesional skin. SJS and TEN are considered to be two ends
of the spectrum of one disease, differing only by their extent of skin detachment.
Management of patients with SJS or TEN requires three measures: removal of
the offending drug, particularly drugs known to be high-risk; supportive mea-
sures and active interventions. Early diagnosis of the disease, recognition of the
causal agent and the immediate withdrawal of the drug are the most important
actions, as the course of the disease is often rapid and fatal.
Keywords: Stevens-Johnson syndrome, toxic epidermal necrolysis, drug eruptions.
Introduction
Stevens-Johnson syndrome (SJS) and toxic epidermal
necrolysis (TEN) are uncommon, acute and potentially
life-threatening adverse cutaneous drug reactions, often
related to drug use. They are the result of extensive death
of keratinocytes, which leads to the separation of areas
of skin in the dermal-epidermal junction, producing the
appearance of scalded skin. The disease runs an unpre-
dictable course: An initially benign-appearing dermato-
sis can progress rapidly.1-5
Stevens and Johnson described in 1922 two cases of
patients with generalized skin rash, continuous fever, sto-
matitis and severe purulent conjunctivitis. In 1950, this
clinical picture was divided into two categories: erythema
multiforme minor (Von Hebra) and erythema multiforme
major (EMM).2,4 As of 1983, the eponymous term Stevens-
-Johnson began to be used interchangeably with EMM.2,4
Only in 1993, Bastuji-Garin et al. proposed that EMM
and SJS would be distinct disorders: EMM would consist
of mucosal erosions and characteristic patterns of cutane-
ous lesions (typical targets, with or without blisters), sym-
metrical and preferably acral distribution, while SJS would
be represented by mucosal erosions and widespread pur-
puric maculae, often confluent, with Nikolsky’s sign posi-
tive and skin detachment limited to less than 10% of the
body surface. EMMwould include recurrent or post-infec-
tious cases or those possibly related to exposure to drugs
with low morbidity and no mortality. SJS, in turn, would
constitute a most serious adverse drug-related disorder with
significant mortality and poor outcome in many cases.4,6
Therefore, although TEN and SJS were historically
considered part of a spectrum of disorders that included
erythema multiforme major, as they all present with mu-
cosal lesions clinically similar, these diseases are now con-
sidered apart. Since the extent of epidermal necrolysis is
a major prognostic factor, it has become a consensus to
classify the spectrum as follows: SJS, cases with skin in-
volvement below 10%; SJS-TEN superposition, cases with