Steven Johnson

Syndrome Toxic
Epidermal Necrolysis
RQA
Reaksi Hipersensitivitas

Reaksi
Hipers
ensitiv
itas

SJS-TEN is essentially drug-
induced. A few cases are
related to infections
(Mycoplasmapneumoniae),
and a few other cases
remain unexplained. HIV
infection dramatically
increases the risk. A
predisposing effect of
autoimmune disorders,
such as systemic lupus, and
an HLA-linked, genetic
susceptibility have been
also suggested.
Klauz Wolf dkk. Fritzpatrick`s Dermatology in General Medicine. 7th ed. McGraw-Hill Companies. USA; 2008.
Diagnosis Banding

Diagnosis Banding
Penisilin termasuk antibiotik -lactam memiliki cincin -lactam
yang secara kimiawi tidak stabil dan terbuka secara spontan
membentuk intermediet reaktif yg berkombinasi dengan grup amino
bebas yg larut atau protein yg berikatan dgn sel membentuk
beberapa antigen, yg paling umum antigen penicilloyl bertindak
sebagai hapten dalam reaksi hipersensitivitas terhadap penicillin
Munir Pirmohamed, PhD. PATHOGENESIS OF PENICILLIN ALLERGY. Journal of the European
Association of Hospital Pharmacists Vol.12 2006/4
Terdapat reseptor apoptotik CD 95 (Fas) di permukaan keratinosit sel T dan NK yg
teraktivasi mengekspresikan ligand Fas (FasL) Fas dan FasL berikatan
menyebabkan apoptosis pd keratinosit di epidermis
Pathogenesis
a) type IVa responses, for which T cells produce interferon (IFN)--
activated macrophages, whose typical clinical manifestation is
eczema; b) type IVb responses, mediated by T cells producing type 2
helper (TH2) cytokines (interleukin (IL) 4 and IL 5, which in turn
induce B cells to produce antibodies and mast cell and eosinophil
responses, mainly in DRESS, MPE, and bullous exanthema; c) type IVc
responses, induced by CD4+ and CD8+ T cells, which produce
cytotoxic mediators that result in keratinocyte apoptosis in MPE and
massive apoptosis in TEN; and d) type IVd responses, characterized by
neutrophil activation and recruitment induced by T cells via the
production of a chemokine, CXCL8, whose typical clinical
manifestation

Patogenesis SJS-TEN di Okular
Aktivasi Fas-FasL di keratinosit + Reaktif limfosit T sitotoksik menginduksi CD4+CD25+ sel T memproduksi cytotoxic
mediators, perforin and granzyme B
Lisis dan apoptosis keratinosit
Terjadi pemisahan dermis dan epidermis
widespread cell death and necrosis in the deep epidermal layers
Dapat diikuti proses infeksi sekunder
Manifestasi pada mata : konjungtivitis kataralis, blefarokonjungtivitis, iritis,
iridosiklitis, kelopak mata edema dan sulit dibuka
Diikuti proses inflamasi pada bulbar and palpebral conjunctiva, but can also involve the cornea, lid margins,
eyelashes andeyelid skin
Prognosis
Bila SCORTEN >3 harus segera mendapatkan perawatan di intensive
care unit