Steven Johnson Syndrome

Steven Johnson Syndrome – Toxic

Epidermal NecrolysisRQA

Reaksi Hipersensitivitas

Reaksi Hipersensitivitas

Klasifikasi Reaksi Alergi

Etiologi• 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

Alergi Penisilin• Penisilin termasuk antibiotik β-lactam memiliki cincin β-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)-γ-

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

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