(2014) Teaching Bangsal - NET

8/12/2019 (2014) Teaching Bangsal - NET

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TOXIC EPIDERMAL

NECROSISTEACHING BANGSAL


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GROUP MEMBERS KHAIRIYAH SURIATMAJAYA NADHIRAH BINTI MOHD. NOH WANDRYATMO SANTOSA TONAPA JIMMY PATABANG SUDARMAN ARUNG T. ANDI DWI RAHMAT ARMYN ANDI ALFISYA BAYU

NURIA IFTITAH DEDIKASIH ANDI TENRI ISMI SHANDRA RACHMAT HIDAYAT


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PATIENTS IDENTITY Name: Ms.Sarmina Gender: Female Age: 40 y.o Marital Status: Married Religion: Islam Address:Jl.Dahlia batangluku kab.gowa Occupation: Housewife Registered: August, 1st 2014


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HISTORY TAKING

Chief complaint:Erotion and scale in whole bodyAnamnesis:

Patient is admitted to RS Wahidin in referralfrom RSUD Syeikh Yusuf with complaint ofgeneralised erotion and scale of whole body withoral lesion, red eyes and tearing as well asgenital laceration since 1.5 month ago. Initially,the patient consumed cefadroxyl three days

before skin manifestation.


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The cefadroxyl was given as a treatment of

the patients ear due to infection witheffusion. There is also complaint ofdifficulty in swallowing food and difficulty

in defecation and urination for more thanone week.The patient is also consulted to otherdepartment which are Internal Medicinedepartment, Opthamology department andENT department.


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TREATMENT

Dexamethasone amp 15 mg/8 hrs/IV Kenalog Ora Base Lanolin 10% + Vaselin 30g Biocream - Face IVFD : RL/NaCl/Dextrose 5% =

1:1:1


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Dermatovenerology status

Location : GeneralisedEfflorescence : scale (+), erosion

(+), crust (+), madidans


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Additional examination:- Complete Blood Count (CBC)August 1st, 2014WBC : 16,3 10 3uLHB :10,71 g/dLAugust 4th, 2014WBC: 19,29 10 3uLHB: 9,1 g/dL


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PRESENT STATUS

General condition : severe illnessConsciousness : composmentisVital sign :BP : 180/70 mmHgPulse : 100x/minute

RR : 32x/iTemperature : 38,4 C


10/38First photo during admission


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Sixth day of treatment


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Diagnosis and Treatment fromInternal Medicine Department

Subdivision : TropicalInfection

Subdivision : GEH

A/ Sepsis ec SSTI A/ GEH

P/ - O2 3 Ltr via nasal canul- Ceftriaxone 2 g/24 hrs/dripsin 100 cc NaCl 0.9%

- Sistenol 3x1

P/ - Diet hepar- HP Pro 1-1-1- Vip Albumin 3x1- Urdahex 250 mg 0-1-1

Additional Exam::SGOT : 32SGPT : 94Ureum : 213Keratin : 1,07


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Diagnosis and Treatment fromOpthamology department

A/ ectropion ODS Tx/ - C. Lyters ODS 6x1 gtt ODS

- C. Polygran 3x1 gtt ODS


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A/ - Acute Tonsillopharyngitis- Otitis Media Externa Profunda

Difus dextra et sinistra

P/ - Oral betadine- Burowi Tampon

Diagnosis and Treatment from ENTDepartment


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Toxic Epidermal Necrolysis (TEN)


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DEFINITION

Toxic Epidermal Necrolysis (TEN) is anacute life-threatening mucocutaneus

reaction which characterized by mucousmembrane erosion, necrolysis, andextensive epidermal detachment.


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The difference between SJS and TEN

is the involvement of body surfacearea (BSA)

< 10% BSA : SJS10-30% BSA : overlapping SJS-TEN

> 30% : TEN


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EPIDEMIOLOGY SJS and TEN are very rare cases. Cases incidence of SJS :

1-6 cases per million persons-years Cases incidence of TEN :

0,4-1,2 cases per million persons-years Occurs at any ages Increasing risk : age after the 4 th decade Women > Men


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ETIOLOGY

Primary cause : DRUGS High risk drugs : sulfonamide antibacterial,

aromatic anticonvulsant, allopurinol, oxicam NSAID, lamotrigine, and nevirapine.

Other etiologies : Mycoplasma pneuomoniae infection, vaccination, graft-versus-hostdisease, and radiation.


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PATHOGENESIS

I mmunologic pattern of early lesion : cell-mediated cytotoxic reaction against

keratinocytes massive apoptosis presence of CD8 T-killer lymphocytes in

dermis and epidermis

CD8 T-killer lymphocyte express - T-cellreceptors and are able to kill through perforinand ganzyme B


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presence of other cytokines, like IL-6, TNF- ,

and Fas-L

I mmunologic pattern of late lesion : >>> monocytes


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CLINICAL MANIFESTATION

Prodromal symptoms (1-14 days): fever, sore throat, chills, headache, malaise

Mucocutaneous lesions : macule that develope into papules, vesicles,

bullae, urticarial plaques, or confluenterythema targetoid lesions


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MUCOSAL LESIONS

M outh : hemorrhage andcrust on lips

erosions in mouthcovered by necroticwhite

pseudomembrane ulcerativestomatitis

Geni tal : painful erosions

Eyes :

erosiveconjunctivitis corneal ulcer


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DIAGNOSIS

1. H istory Taking

- Non-specific symptom (1-3 days) : fever,stinging eyes, pain upon swallowingheadache, rhinitis, myalgia.- Epidermal necrolysis : 8 weeks after drugsconsumption- Initial lesion : erythema macula (bodytrunks, then spread to other body sites)


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2. Physical Examination Skin :

- irregular-shaped erythema,hyperpigmentations, purpuric macules- targetoid lesion- confluent necrotic lesions- Nikolsky sign (+)- lesions evolve into flaccid blisters


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Mucous membrane:

- buccal, ocular, genital mucous- painful erythema and erosion- impaired function (impaired alimentation, photophobia, conjunctival synechiae,dysuria)


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3. Laborator ium Examination Blood Examination

- anemia - lymphopenia-leukocytosis - mild thrombocytopenia

- eosinophilia - neutropenia- blood urea nitrogen - serum urea - electrolyte imbalance - serum bicarbonate

- hypoalbuminemia - hypoproteinemia


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Immunologic Examination- perifer CD4 lymphopenia

4. Dermatopathology Examination

- sparse apoptotic keratinocytes (suprabasallayers) which rapidly evolve to a full-

thickness EN and sub-epidermal detachment- mononuclear cell infiltrate


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- among T cells : lymphocytes CD8 with phenotypic features of cytotoxic cells

- eosinophils : less common in patients with

the most severe form of TEN.


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SCORTEN (Score of Toxic Epidermal Necrolysis )

Fitzpatricks Dermatology in General Medicine 8th Edition


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THERAPY

Early recognition Prompt withdrawal of the offending drugs

1. Symptomatic Treatment Only patients with limited skin involvement and

SCORTEN score of 0 or 1. Manage replacement of IV fluid and electrolytes Nutrition support (nasogastric tube)


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Environmental temperature : 28C 30C Eyes : - examined daily by ophtalmologist

- artificial tears, antibiotic or antisepticeyedrops, vitamin A, mechanicaldisruption of early synechiae

Pruritus : antihistamine


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2. Specif ic Treatment High-dosed IV IG : 0,75 mg/kg/day for 4

consecutive days Cyclosporin : 3 mg/kg/day Plasmapharesis or Hemodialysis : prompt the

removal of the offending drugs, itsmetabolites, or inflammatory mediators.


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Corticosteroid :- controversial- given in early phase- dexamethasone 40 mg/day

Cyclosporine A- powerful immunosuppressive agent

- activates of T helper 2 cytokines, inhibitionof CD8+ cytotoxic mechanisms, andantiapoptotic effect


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DIFFERENTIALDIAGNOSES


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SJS TENERYTHEMA

MULTIFORMS.S.S.S

PEMPHIGUSVULGARIS

Etiology

drugs, infection,vaccination, graft-

versus-host disease,radiation

drugs, infection,vaccination, graft-

versus-hostdisease, radiation

autoimmune,drugs, viral or

bacterial infection

Staphylococcus

aureus

genetic,

autoimmune

ClinicalFeatures

macule, papule, plaque, erosion,ulcer, necrosis,

blistering,conjunctivitis

macule, papule, plaque, erosion,ulcer, necrosis,

blistering,conjunctivitis,

epydermolysis, Nickolsky sign (+)in erithematous

area

erythematousmacule, well-marginated,

edematous papule,iris lesion

desquamation,skin exfoliative in

sheets, lesionsmore superficial

(granular layer), Nickolsky sign(+)

mucous erosionand thin-walled,

flaccid, easilyrupture bullae,

Nickolsky sign(+)

Predilection trunk, palms, soles trunk, palms, soles

dorsal hands

symmetrically,acrally

neck, groin,axillae

mouth, burn orskin injury sites

Age

increasing risk atage > 40 y.o

woman > man

increasing risk atage > 40 y.o

woman > man

adolescence neonates &children

middle age

woman = man


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COMPLICATIONS

Acute phase : sepsis is the most commoncomplication

Respiratory system : bronchopneumonia Genitourinary system : acute tubular necrosis,

renal failure, penile scar, or vaginal stenosis Gastroenterology system : esophageal stricture Ocular system : corneal ulcer, anterior uveitis,

panophtalmitis, blindness


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PROGNOSIS

SJS TEN : life-threatening diseases Overall hospital mortality of TEN is 22-25%

Mortality rate of SJS : 5-12% Mortality rate of TEN : >30%

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(2014) Teaching Bangsal - NET