Sunarsih, S.Kep., Ns

ASKEP KLIEN DENGAN GANGGUAN SISTEM INTEGUMEN(STEVEN JHONSON SYNDROME)

What is Steven Jhonson Syndrome

• Stevens-Johnson syndrome (SJS) is an immune-complex–mediated hypersensitivity complex that is a severe expression of erythema multiforme.

• Stevens-Johnson syndrome (SJS) is a potentially deadly skin disease that usually results from drug reaction.

• Another form of the disease is called Toxic Epidermal Necrolysis (TEN), and again this usually results from a drug related reaction.

• SJS typically involves the skin and the mucous membranes. While minor presentations may membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis.

Criteria

• A clasification was developed for epidemological purpose to distinguish between Steven-Jhonson Syndrome and Toxic Epidermal Necrolysis (TEN)

• This classification proposes that SJS is less than 10% of surface area of skin is involved

• Greather than 10% - 29% is called SJS-Ten overlap• 30% or greater skin surface involvement is considered TEN

Causes of Steven Jhonson Syndrome

• Drugs that have been linked to steven jhonson syndrome include NSAIDS (Non steroid anti inflamatory drugs)

• Antibiotik• Anti piretik dan analgetik• Anti convulsan• In many cases there is known cause for the onset of steven jhonson syndrome or

toxic epidermal necrolysis• Drugs cause over 80% of all cases of SJS &TEN• 90-95% of TEN cases are caused only by medication

Pathophysiology

• SJS is an immune-complex–mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome.

• Pathologically, cell death results causing separation of the epidermis from the dermis

• Drugs and malignancies are most often implicated as the etiology in adults and elderly persons.

• Pediatric cases are related more often to infections than to malignancy or a reaction to a drug.

• Various carcinomas and lymphomas have been associated.

Symptoms of Steven Jhonson Syndrome

• Choughing • Aching• Headaches• Vomiting• Skin lession (painful), skin blister• Mucous membran lession• Mouth rash, mouth blister• Throat rash, troath blister• Anus rash, anus blister• Genital rash, genital blister• Conjungtival rash

• Conjungtival blister• Target shaped blister on the eyes• Skin rash• Tiredness• Fever• Malaise• Burning sensation• Joint pain• Muscle pain• mucous membran ulcers• Painful skin blister• Painful conjungtivitis

Management of SJS

Diagnosis: biopsy

Removal of cause

Spesific treatment

Complications management

Fluid, electrolyte, infection

Wound care, Pain

management

Complications of SJS

• Conjungtivitis • Iritis• Corneal blister• Corneal erosions• Corneal holes• Corneal scarring• Loss of vision• Death

Prognosis

Pada kasus yang tidak berat, prognosisnya baik, dan penyembuhan terjadi dalam waktu 2-3 minggu. Kematian berkisar antara 5-15% pada kasus berat dengan berbagai komplikasi atau pengobatan terlambat dan tidak memadai. Prognosis lebih berat bila terjadi purpura yang lebih luas. Kematian biasanya disebabkan oleh gangguan keseimbangan cairan dan elektrolit, bronkopneumonia, serta sepsis.

Pengkajian

• Identitas klien• Riwayat kesehatan• Status kesehatan saat ini• Pemeriksaan fisik

Patient history• Patient admitted with c/o redness and itching of eyes since 6days.• Itchy red lesions,forearm,neck since 6days.• Dysuria since 6days.• Multiple hyperpigmented macules over forearm,chest,dorsum of both

hands,palms,ears.• Erotion + over labia majora,labia minor,clitoris.

Nursing Diagnosis

• Acute pain related to chemical injury agent• Impaired skin integrity related to medication side effects• Impaired oral mucous membrane related to medication side effects• Imbalance nutrition: less than body requirement related to inability to

ingest food• Disturbed body image related to illness• Risk for infection related to inadequate primary defenses (impaired

skin integrity)• Defisit self care syndrome related to illness• Deficient knowledge related to unfamiliarity with information resorces

Nursing Intervention

Acute pain related to chemical injury agent• NOC

- Comfort level- Pain control- Pain level

• NIC- Pain management- Analgesic administration- Anxiety reduction- Enviromental management: comfort

Impaired skin integrity related to medication side effects• NOC

- Tissue integrity: skin & mucous membrane- Wound healing: Primary intention

• NIC- Skin survailance- Wound care- Potitioning- Medication administration- Fluid/electrolyte management

Impaired oral mucous membrane related to medication side effects• NOC

- Oral hygine- tissue integrity: skin&mucous membrane

• NIC- Oral health maintenance- Oral health promotion- Oral health restoration

Imbalance nutrition: less than body requirement related to inability to ingest food

• NOC- Nutritional status- Nutritional status: food&fluid intake- Nutritional status: nutrient intake

• NIC- Nutritional monitoring- Nutritional counseling- Nutritional management

Disturbed body image related to illness• NOC

- Adaption to physical disability- Body image- Child development: Adolscence- Self-Esteem

• NIC- Body image enchacement- Coping enchacement- Emotional support

Risk for infection related to inadequate primary defenses (impaired skin integrity)

• NOC- Immune status- Knowledge: infection control- Risk control- Nutritional status

• NIC- Infection protection - Nutritional management- infection control - Enviromental management- Exercice promotion- Wound care

Deficient knowledge related to unfamiliarity with information resorces • NOC

- Knowledge: ilness care- Knowledge: treratment procedure- Knowledge: medication- Knowledge: diet

• NIC- Teaching disease process- Teaching prescribe diet- Teaching prescribe medication- Teaching prescribe exercise