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Ocular sequelae require daily examinations by an oph-
thalmologist.
Active interventions
Specific therapies for SJS and TEN have not yet reached
evidence-based acceptance standards. The low prevalence
of the disease and its lethal potential make it difficult to
perform randomized clinical trials.
Some reviews concluded that steroids do not short-
en the duration of disease and may also increase the risk
of infections and worsen healing. Many authors do not
recommend the routine use of systemic steroids in the
treatment of SJS/TEN but some centers advocate an ear-
ly pulse (first 48 hours).
13-17
Studies have suggested benefit of plasmapheresis for
the treatment of SJS/TEN; however, there are reports
showing that its use did not significantly affect mortali-
ty and length of hospital stay in some cases.
13-17
Cyclosporin is an immunosuppressive medication
with anti-apoptotic activity and has been considered as
a potentially useful drug for treatment; however, its use-
fulness is not well defined.
13-17
Viard et al., in 1998, reported that commercial prep-
arations of intravenous immunoglobulin contained nat-
ural anti-Fas (anti-CD95) antibodies that blocked Fas to
FasL binding, thus intervening in disease pathogenesis.
The studies show mixed results. Successful treatment de-
pends on the dose and its early use.
13-17
P
rognosis
Prognosis is linked to rapid identification of the causative
drug and its discontinuation. It is crucial to quickly estab-
lish proper clinical diagnosis, so that the causative drug
may be discontinued and appropriate treatment initiated.
In SJS, the mortality rate is typically less than 5%, and
sepsis is the main cause of death. Prognosis does not seem
to be affected by the type or the dose of the causative drug,
or by HIV infection.
A score called SCORTEN developed by Bastuji-Garin
et al. determines the variables as predictors of prognosis
and risk of death in patients with SJS and TEN.
18
In addition to the SCORTEN predictors, other fac-
tors determinant of poor prognosis include late with-
drawal of the causative drug and delay to transfer the pa-
tient to an aseptic or burn unit.
R
esumo
Síndrome de Stevens-Johnson e necrólise epidérmica tó-
xica: revisão
A síndrome de Stevens-Johnson (SSJ) e a necrólise epidér-
mica tóxica (NET) são doenças mucocutâneas pouco fre-
quentes, agudas e potencialmente ameaçadoras à vida.
Representam uma reação de hipersensibilidade e podem
ser desencadeadas por fármacos, infecções e neoplasias.
Dentre os principais medicamentos descritos como cau-
sadores do quadro estão o alopurinol, alguns antibióti-
cos do grupo das sulfonamidas, anticonvulsivantes, como
carbamazepina, e alguns anti-inflamatórios não esteroi-
dais. A necrose dos queratinócitos manifesta-se clinica-
mente pelo descolamento epidérmico, levando a um as-
pecto de pele escaldada. A erupção inicia-se no tronco,
com posterior generalização, geralmente poupando as
áreas palmoplantares. As máculas tornam-se violáceas e
há descolamento epidérmico, dando origem a bolhas flá-
cidas, que confluem e se rompem, deixando áreas exten-
sas erosadas. A pele perilesional apresenta sinal de Nikol-
sky positivo. A SSJ e a NET representam espectros da
mesma doença, diferenciando-se pelo grau de descola-
mento epidérmico. O tratamento da SSJ e da NET é fun-
damentado em três medidas: retirada da droga ofensora,
especialmente as medicações conhecidamente de alto ris-
co; medidas de suporte e intervenções ativas. O diagnós-
tico precoce da entidade, o reconhecimento do agente
causal e a retirada imediata do fármaco são as mais im-
portantes ações, visto que a evolução dos casos é muitas
vezes rápida e fatal.
Palavras-chave:
síndrome de Stevens-Johnson, necróli-
se epidérmica tóxica, erupção por droga.
R
eferences
1. Oliveira A, Sanches M, Selores M. [Stevens-Johnson syndrome and toxic
epidermal necrolysis]. Acta Med Port. 2011; 24 Suppl 4:995-1002.
2.
Ellender RP, Peters CW, Albritton HL, Garcia AJ, Kaye AD. Clinical
considerations for epidermal necrolysis. Ochsner J. 2014; 14(3):413-7.
3.
Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis:
clinical patterns, diagnostic considerations, etiology, and therapeutic
management. Semin Cutan Med Surg. 2014; 33(1):10-6.
4. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part I.
Introduction, history, classification, clinical features, systemic manifestations,
etiology, and immunopathogenesis. J AmAcadDermatol. 2013; 69(2):173.e1-13.
5.
Lee HY, Chung WH. Toxic epidermal necrolysis: the year in review. Curr
Opin Allergy Clin Immunol. 2013; 13(4):330-6.
6. Tomasini C, Derlino F, Quaglino P, Caproni M, Borroni G. From erythema
multiforme to toxic epidermal necrolysis. Same spectrum or different
diseases? G Ital Dermatol Venereol. 2014; 149(2):243-61.
7.
Bulisani ACP, Sanches GD, Guimarães HP, Lopes RD, Vendrame LS, Lopes
AC. Síndrome de Stevens-Johnson e necrólise epidérmica tóxica emmedicina
intensiva. Rev Bras Ter Intensiva. 2006; 18(3):292-7.
8.
Dodiuk-Gad RP, Laws PM, Shear NH. Epidemiology of severe drug
hypersensitivity. Semin Cutan Med Surg. 2014; 33(1):2-9.
9.
Naldi L, Crotti S. Epidemiology of cutaneous drug-induced reactions. G
Ital Dermatol Venereol. 2014; 149(2):207-18.
10. Thong BY. Stevens-Johnson syndrome / toxic epidermal necrolysis: an Asia-
Pacific perspective. Asia Pac Allergy. 2013; 3(4):215-23.