Multazam C014182120

Ghina Adila Hafizhah C014182216

Muhammad Fikri Hidayat C014182243
Nadya Juliani Rusdi C014182245
Nanda Ayuni Mutmainnah C014182247

dr. Irma Herlina , Sp.KK

dr. Rizka Ramadhani Ruray
Stevens-Johnson syndrome (SSJ) is a form of mucocutaneous
disease with severe systemic signs and symptoms in the form
of target lesions with irregular shape, accompanied by
macules, vesicles, bullae, and purpura which are widespread,
especially in the skeleton of the body.
Drugs are the main etiology that can occur in adults or children.

Top three highest sequences of drugs suspected of causing SSJ and NET :
1. Analgesic drugs (32.86%)
2. Antibiotics (20.14%)
3. Antiepileptic drugs (15.9%).
1. History
2. Cutaneous Lessions & Mucous
membrane involvement
3. Systemic Involvement
1. Erhytema Multiforme Majus
2. Varicella
3. Acute Generalized Exanthematous Pustolosis
4. Generalized Bullous Fixed Drug Eruption
5. Staphylococcal skin syndrome
- Biopsy
- Immunofluorescence examination
 Variable Score
Age >40 years 1
Heart rate >120x/minutes 1
Malignancy 1
Initial precentage of epidermal detachment >10% 1
Blood urea nitrogen (BUN) level >10mmol/L 1
Bicarbonate >20mmol/L 1
Serum glucose level >14mmol/L 1
SCORTEN score Mortality (%)
0-1 3.2
2 12.1
3 35.8
4 58.3
5 90
 Drugs adverse reaction

Immune Non-immune Idiosyncratic

mechanisms mechanisms mechanisms

Genetic: HLA-B 1502, HLA-B 5801 Epidermal

Immune : Type IV hypersensitivity response Necrolysis

SA Martínez-Cabriales, News in severe clinical adverse drug reactions: Stevens-Johnson syndrome (SJS) and toxic
epidermal necrolysis (TEN). Gaceta Médica de México. 2015;151: 721-31.
Four models of the interaction of human leukocyte antigen (HLA), drug and T-cell
receptor (TCR)

Chung, W.-H., Wang, C.-W., & Dao, R.-L. Severe cutaneous adverse drug reactions. The Journal of Dermatology, 2016.
43(7), 758–766
Cytotoxic proteins and cytokines/chemokines in SJS/TEN

Su, S.-C., & Chung, W.-H.Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins,
2014. 6(1), 194–210
Granulysin is secreted via a non-granule exocytotic pathway, which
enables for it to enter in the keratinocyte and cause cell death by means of
damage to the cell membrane and disruption of the mitochondrial
transmembrane potential.
The idea demonstrates that the Fas activation may act as a death receptor from
FasL recognition. The Fas–FasL complex binds to Fas-associated death domain
protein (FADD) which concurrently recruits procaspase 8 in a manner of multiple
copies. They subsequently autoactivate from procaspase 8 to caspase 8, initiating a
caspase cascade that results in intracellular DNA degradation.
Reported that the inhibition of perforin and granzyme B
expression is able to relieve the cytotoxicity of SJS/TEN blister
keratinocytes, whereas it did not have the inhibition reaction by
the anti-Fas antibody.

1. Wong A, Malvestiti AA, Hafner Mde F. Stevens‐Johnson syndrome and toxic epidermal necrolysis: a review. Rev Assoc Med Bras.
2016(62):468–73.
2. Chung, W.-H., Wang, C.-W., & Dao, R.-L. Severe cutaneous adverse drug reactions. The Journal of Dermatology, 2016. 43(7), 758–766
3. Su, S.-C., & Chung, W.-H.Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins, 2014. 6(1), 194–
210
Binding of TNF-α to its cell surface receptor triggers apoptosis
through DISC-mediated activation of caspase cascade and
mitochondrial changes, leading to a series of cytotoxic processes,
including generation of free radicals and damage to nuclear DNA by
endonucleases.

1. SA Martínez-Cabriales, News in severe clinical adverse drug reactions: Stevens-Johnson syndrome (SJS) and toxic epidermal
necrolysis (TEN). Gaceta Médica de México. 2015;151: 721-31.
2. Su, S.-C., & Chung, W.-H.Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins, 2014. 6(1),
194–210
IFN-γ orchestrates the cytotoxic activities, to some extent, by
induction of ROS, which is a shared mechanism connecting the
involvement of IFN-γ in SJS/TEN with TNF-α and FasL.

1. SA Martínez-Cabriales, News in severe clinical adverse drug reactions: Stevens-Johnson syndrome (SJS) and toxic epidermal
necrolysis (TEN). Gaceta Médica de México. 2015;151: 721-31.
2. S.-C., & Chung, W.-H.Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins, 2014. 6(1), 194–
210
IL-2, IL-5, IL-6, IL-10, IL-12, IL-13, IL-15, IL-18, CCR3, CXCR3, CXCR4, and
CCR10.

1. Chung, W.-H., Wang, C.-W., & Dao, R.-L. Severe cutaneous adverse drug reactions. The Journal of Dermatology, 2016. 43(7), 758–
766.
2. S.-C., & Chung, W.-H.Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins, 2014. 6(1), 194–
210
Thank You 