Anda di halaman 1dari 29

Apa yang terjadi pada kulit anakku?

Oh tidaaaakk!
Identitas
Nama An. SRCP
Umur 8 tahun 9 bulan
Alamat Ngepringan 9/1, Pagutan, Sukoharjo
Jenis Perempuan
kelamin
No RM 375842
Tanggal 05-11-2017 (21:21)
masuk
Tanggal 05-11-2017
Periksa
Anamnesis

Keluhan utama : ruam kemerahan pada kulit


Riwayat Penyakit Sekarang

Pasien datang dibawa keluarga rujukan dari salah satu RS


dengan erupsi obat. Pasien sebelumnya memliki riwayat
epilepsi sejak usia 3 tahun. Sebelumnya rutin mengonsumsi
obat anti kejang namun masih sering kambuh, riwayat
pengobatan rutin diazepam, as. Valproat, fenobarbital,
fenitoin dan terakhir 10 hari yll carbamazepin. Kemarin pagi
pasien kontrol karena demam dan diberikan cefixime baru
diminum 1x pagi, siang harinya mulai muncul kemerahan di
dada dan perut. obat tidak dilanjutkan dan pasien langsung
dibawa ke SpA tsb, kemudian dirujuk ke RSUD Sukoharjo
dikarenakan di RS sebelumnya tidak terdapat SpKK.
Riwayat Penyakit Dahulu

- Riw. epilepsi sejak usia 3 tahun


- Alergi penisilin (+)
Riwayat Penyakit Keluarga

-DM (-)
-Hipertensi (-)
-Alergi (-)
-Keganasan (-)
Pemeriksaan Fisik

Keadaan Umum : Lemas


Kesadaran : Compos Mentis
Tanda Vital
-TD : 110/70 mmHg
-N : 102 x/menit
-RR : 24 x/menit
-Suhu :37,9 C
Kepala
• Bentuk : Bulat, Normocephal, Deformitas (-)
• Mata : Konjungtiva Anemis (-/-), Sklera Ikterik (-/-)
• Telinga : Discharge (-/-). Nyeri Tekan (-/-)
• Hidung : Deviasi Septum (-), Perdarahan (-), Sekret (-)
• Leher : Bentuk normal, deviasi trakea (-),
pembesaran KGB (-)
Paru
• Inspeksi : Normochest, Simetris Kanan=kiri
• Palpasi : Ekspansi Dada Simetris, Fremitus Taktil (+/+)
• Perkusi : Sonor
• Auskultasi : Vesikuler (+/+), Ronki (-/-), Wheezing (-/-)

Cor
• Inspeksi : ictus cordis tidak tampak
• Palpasi : ictus cordis tidak teraba
• Perkusi : batas kanan jantung ICS IV parasternal dextra,
batas kiri ICS IV midclavicula sinistra, batas atas ICS III
parasternal sinistra
• Auskultasi : BJ I/II reguler, gallop (-), murmur (-)
Abdomen
• Inspeksi : cembung, makula eritem multipel diskret
• Auskultasi : BU (+) (N), Hiperistaltik (-), Metallic Sound (-)
• Palpasi : Supel, Nyeri tekan (-)
• Perkusi : Timpani (+)

Ekstremitas
Atas : Akral hangat (+/+), edema (-/-), capillary refill < 2 detik,
makula eritem multipel diskret
Bawah : Akral dingin (+/+), edema (-/-), capillary refill < 2 detik,
makula eritem multipel diskret
Pemeriksaan Penunjang

Parameter Hasil Referensi


Hematologi
-Hb 11.4 10 – 16
-Hct 33.4 33 – 38
-AL 5.8 9 – 12
-AT 154 150 – 500
-AE 3.88 4–5
SCORTEN

SCORTEN belum bisa dilakukan


Karena belum ada intruksi
cek ureum, bikarbonat, dan
gula darah
Diagnosis

-Epilepsi
-Drug eruption susp SJS ec carbamazepin dd
cefixime
Tatalaksana IGD
Tx SpA
Inf. RL 24 tpm
As. Valproat 2x300mg
Diazepam 3x2,5mg
Paracetamol 2cth /5jam kp demam
Ranap
Raber SpKK

Tx SpKK
Inj. MP awal di IGD 0,5cc dilanjutkan
Inj. MP pagi (07.00) 0,5cc
siang (14.00) 0,3cc
Inj. Ranitidin 0,5cc /12jam
Cetirizin 2x1/2 tab
Follow Up
06-11-2017
Tx SpA
Inf. RL 24 tpm
As. Valproat 2x300mg Tx SpM
Diazepam 3x2,5mg Cenfresh 3x1 tts ODS
Paracetamol 2cth /5jam kp demam Tobroson 4x1 tts ODS
Raber SpKK & SpM

Tx SpKK
Inj. MP pagi (07.00) 0,5cc
siang (14.00) 0,3cc
Inj. Ranitidin 0,5cc /12jam
Cetirizin 2x1/2 tab
Exton C 0,5cc/hari
Eritroma syr 3x500mg
Definition

 SJS/TEN:
 Lesions: Small blisters on dusky purpuric macules or
atypical targets
 Mucosal involvement common
 Prodrome of fever and malaise common
 Stevens-Johnson Syndrome:
 Rare areas of confluence.
 Detachment </= 10% BSA
 Toxic Epidermal Necrolysis:
 Confluent erythema is common.
 Outer layer of epidermis separates easily from basal layer
with lateral pressure.
 Large sheet of necrotic epidermis often present.
 >30% BSA involved.
Presentation

 Fever (often >39) and flu-like illness 1-3 days


before mucocutaneous lesions appear
 Confluent erythema
 Facial edema or central facial involvement
 Lesions are painful
 Palpable purpura
 Skin necrosis, blisters and/or epidermal detachment
 Mucous membrane erosions/crusting, sore throat
 Visual Impairment (secondary to ocular
involvement)
 Rash 1-3 weeks after exposure, or days after 2nd
exposure
Epidemiology

 2-7/million people/year  Risk Factors:


 SJS: age 25-47, TEN: age 46-  HIV infection
63  Genetic factors
 Women: >60%  Certain HLA types
 “Slow acetylators”
 Poor prognosis:
 Polymorphisms in IL4 receptor
 Intestinal/Pulmonary gene
involvement
 Concomitant viral infections
 Greater extent of detachment
 Underlying immunologic
 Older age diseases
 Mortality:  Physical factors
 SJS: 5%  UV light, radiation therapy
 TEN: 30%  Malignancy
 Higher doses of known
offenders
Pathogenesis

 Secondary to cytotoxicity and delayed hypersensitivity


reaction to the offending agent.
 Antigen is either the implicated drug or a metabolite.
 Histopathology:
 Granulysin (cytolytic protein produced
by cytotoxic T cells and NK cells)
 Expression of HLA-DR and
intracellular adhesion molecule
(ICAM)-1 by
 Keratinocytes
 CD4 cells (in dermis)
 CD8 T cells (in epidermis)
 Apoptosis of keratinocytes
facilitated by Subepidermal split with cell-poor bullous.
 TNF-alpha, perforin and granzyme Epidermis shows full thickness necrosis.
secretion
 fas-ligand expression (cell death receptor)
Etiologies

 Medications (Odds Ratio for exposure in hospitalized


pts):
 Sulfonamide antibiotics (172)
 Allopurinol (52)
 Amine antiepileptics
 Phenytoin (53)
 Carbamazepine (90)
 Lamotrigine
 NSAIDs (72)
 Infections (e.g. Mycoplasma pneumonia)
 Other: Vaccinations, Systemic diseases, Chemical
exposure, Herbal medicines, Foods
Differential Diagnosis for Vesicular or Bullous Rash

Bullous
Pemphigoid
Often affects
the elderly

Dermatitis Herpetiformis
Associated with gluten intolerance

Pemphigus
Affects middle-aged or elderly

Cicatricial Pemphigoid
Mucosal involvement, sometimes cutaneous
Differential Diagnosis, cont.

Linear IgA Disease


Itchy, ring-shaped, no internal disease

Herpes Simplex Virus

Varicella/Zoster Virus

Hand-Foot-Mouth
Disease
(Enteroviruses) Contact Dermatitis
Differential Diagnosis, cont.
 Erythema Multiforme
 Staphylococcal Scalded Skin Syndrome
 Bullous Impetigo
 Toxic Shock Syndrome
 Paraneoplastic Pemphigus
 Cutaneous emboli
 Diabetic Bullae
 Porphyria Cutanea Tarda
 Porphyria Variegata
 Pseudoporphyria
 Bullous dermatosis of Hemodialysis
 Coma Bulloae
 Epidermolysis Bullosa Acquisita
Treatment

 Early diagnosis - biopsy


 Immediate discontinuation of offending agent
 Supportive care – pay close attention to ocular
complications
 IV hydration (e.g. Parkland formula)
 Antihistamines
 Analgesics
 Local v. systemic corticosteroids
 Think about nursing requirements!
 Possible treatment in burn unit, wound care
 IVIg?
Prognosis

Independent Prognosis Factors Weight


Age >/= 40 years 1
Malignancy Yes 1
BSA Detached >/= 10% 1
Tachycardia >/= 120/min 1
Serum urea >10 mmol/l 1
Serum glucose >14 mmol/l 1
Serum bicarbonate <20 mmol/l 1
SCORTEN # 7
Resources:

 Cooper, et al. The Washington Manual of Medical


Therapeutics, 32nd Edition. 2007.
 High, et al. Stevens-Johnson syndrome and toxic
epidermal necrolysis: Management, prognosis, and
long-term sequelae. Up To Date. 2009.
 Kasper, et al. Harrison’s Principles of Internal
Medicine, 16th Edition. 2005.
 Nirken, et al. Stevens-Johnson syndrome and toxic
epidermal necrolysis: Clinical manifestations,
pathogenesis, and diagnosis. Up To Date. 2009.
MATUR NUWUN