Steven johnson syndrome & ten

STEVEN-JOHNSON SYNDROME & Toxic Epidermal Necrolysis

Prof DR Dr Ariyanto Harsono SpA(K)

Difinition

Steven-Johnson Syndrome (SJS) is a collection of clinical symptoms of mucocutaneous eruption characterized by a triad of abnormalities in vesicobulous skin, eyes and mucous orifice accompanied by severe general symptoms. Synonyms include: de Friessinger-Rendu syndrome, erythema exudativum multiform major, Poliform bullous erythema, Muko-Cutaneo-ocular syndrome, dermatostomatitis

2Prof DR Dr Ariyanto Harsono SpA(K)

PathophysiologySJS etiology is difficult to determine with certainty, because

the cause of various factors, although in general is often associated with immune response to drug. Some of the factors causing SJS include: infection (viral, fungal, bacterial, parasitic), drugs (Vaccine, salicylates, sulfa, penicillin, ethambutol, Tegretol, tetracycline, digitalis, contraceptives), food (chocolate), physical (cold air, sunlight, X-ray), miscellaneous (collagen disease, malignancy, pregnancy).


Pathophysiology…..

Pathogenesis of SJS is often associated with type III hypersensitivity reactions (immune complex reaction) induced by soluble complexes of antigen or its metabolites with IgM and IgG antibodies and delayed-type hypersensitivity reactions ( hypersensitivity reactions type IV) is a reaction that is mediated by specific T lymphocytes.

Prof DR Dr Ariyanto Harsono SpA(K) 4

Clinical Manifestation

Prodromal symptoms ranges from 1-14 days of fever, malaise, cough, coryza, pain swallowing, chest pain, vomiting, muscle aches and arthralgia vary greatly in severity and combinations of symptoms.


Clinical Manifestation…..

After that will arise in the lesions:basic lesion is Erythema Exudativum multiforme, over 10% of

the body surface. There are several "target lesion" is a pathognomonic symptom.

Form of skin erythema, papul, vesicles, or bullae symmetrically on almost the entire body.

Mucosa in the form of vesicles, bullae, erosion, excoriation, bleeding and red crustae. Bullae occur suddenly within 1-14 days prodromal symptoms, appear on mucous membranes, membranes of the nose, mouth, anorectal, vulvovaginal area and urethral meatus. Ulcerative stomatitis and crusting hemorrhagic is a prominent feature.


Target Lesion


Clinical Mabifestation…..

Eyes: catarrhalis conjunctivitis, blepharoconjunctivitis, iritis, iridocyclitis, eyelid edema and difficult to open, in severe cases erosion and perforation of the cornea that can lead to blindness. Ocular mucosa injury is the trigger that causes ocular cicatricial pemphigoid, a chronic inflammation of the ocular mucosa which causes blindness. The time required from onset until the occurrence of ocular cicatricial pemphigoid vary from a few months to 31 years.


Clinical Manifestation…..

The basis of skin lesions is erythema exudativum multiforme (EEM).

When extensive EEM <30% is called Erythema Exudativum minor,

if> 30% Exudativum: Erythema multiforme major, when accompanied by abnormalities in more than 2 mucosa:

Steven Johnson Syndrome (SJS). SJS be accompanied bullae, when <10% is called Steven Johnson Syndrome, when 10-30% bullae called Steven Johnson Syndrome-Toxic

Epidermal-Necrolisis (SSJ-TEN), when the bullae> 30% is called Toxic Epidermal Necrolisis

(TEN).10Prof DR Dr Ariyanto Harsono SpA(K)

Basic lesion is erythema exudativum multiforme. Looks some "target lesion".


Mucosal Lesion at mouth, eyes, genitals, and maybe anal(minimal 3 mucosa)


Diagnosis

Diagnosis directed against manifestations according to the triad of skin disorders, mucosa, eyes, and its relationship with the pathognomonic target-shaped lesions, or eye iris cattle, mucosal abnormalities, fever. Also supported laboratory tests such as examination of peripheral blood, immunological examination, bacterial culture and test the peripheral blood, and histopathological examination of the skin biopsy.


Diagnosis…..

Anemia can be found in severe cases with bleeding, leukocytes usually normal or slightly elevated, increase in eosinophils. Levels of IgG and IgM elevate, C3 and C4 normal or slightly decrease circulating immune complexes. A skin biopsy: there is no classic abnormality. Imunoflurosesensce can help diagnose of atypical

cases.


Differential Diagnosis

• The main differential diagnosis is Staphylococcal scalded skin syndrome (4S) and

• Toxic epidermal necrolysis (TEN) which is almost similar clinical manifestations but the general state of TEN looks worse than SJS. TEN is the fact of heavy SJS.


Staphylococcal Scalded Skin Syndrome


Staphylococcal Scalded Skin Syndrome…..


Toxic Epidermal Necrolysis


TEN…..


Management

In general, patiants with SJS come up in general in severe condition that therapy is usually given late:

Fluids and electrolytes, protein and calories parenterally.

Broad-spectrum antibiotics, based on culture results and subsequent resistance test preparation germs of the skin lesions and blood.


Management…..

• Coticosteroid parenteral: dexamentasone initial dose of 1mg/kg bolus, then for 3 days 0.2-0.5 mg / kg every 6 hours. The use of systemic steroids is still controversial, there is assume that the use of systemic steroids in children can lead to slow healing and significant side effects, but some are considered favorable steroid and save lives.


Management…..

Antihistamines if necessary. Especially when there is intense itching. Pheniraminehydrogen maleate (Avil) can be given at a dose for ages 1-3 years 7.5 mg / dose, for ages 3-12 years 15 mg / dose, administered 3 times / day. As for the dose of cetirizine can be given to children aged 2-5 years: 2.5 mg / dose, 1 time / day;> 6 years: 5-10 mg / dose, 1 time / day.

Eyes as well as skin care and Topical antibiotics


Management…..

Bullae in skin treated with wet compresses of Burowi solution.

Not allowed to use topical steroids on skin lesions.

Mouth Lesions were treated with Kenalog in Orabase.

Secondary infection treated with antibiotic therapy that rarely cause allergies, broad spectrum, bactericidal and is not nephrotoxic, such as intravenous clindamycin 8-16 mg / kg / day intravenously, given 2 times / day.


Prognosis

In the cases that are not severe, the prognosis is good, and healing occurs within 2-3 weeks. Mortality ranged from 5-15% in severe cases with complications or late and inadequate treatment. Prognosis is more severe if there is a wider purpura. Death is usually caused by disorders of fluid and electrolyte imbalance, bronchopneumonia and sepsis.


Toxic Epidermal Nekrolisis (TEN)

Toxic epidermal necrolysis, also known as TEN, is a potentially deadly skin disease that usually resulting from drug reactions. Such as Stevens-Johnson Syndrome, the disease is usually a reaction to medication. Both forms of the disease can be deadly and extremely painful and distressing. Other drugs associated with toxic epidermal necrolysis is penicillin.

In many cases there is no known cause for TEN, although the drug is the major cause.


This condition can be very serious, and can cause discomfort and pain, and in some cases the infection can be spread through the disease result in death of the patient.

Every age group can be affected by toxic epidermal necrolysis. However, usually seen in older patients than younger ones, and this is because older patients are more likely to take medication, and therefore more likely to find that they have a drug reaction. There are also other groups that are more vulnerable when it comes to acquiring toxic epidermal necrolysis. This includes those with AIDS, who have low immunity to infection and disease.

Parents and all other age groups are advised to be alert TEN signs if they are in treatment.


Clinical Manifestation

Both toxic epidermal necrolysis and SJS can begin with non-specific symptoms such as cough, pain, headache, and fever. This may be followed by symptoms more specific like a red rash on the face and trunk, which can continue to spread to other parts of the body. The rash can form into blisters, and blisters may form in areas such as the eyes, mouth and vaginal area. Mucous membranes can be inlflammed.TEN is heavy SJS. Bullae on TEN wider than 20%, a positive sign Nichosky may be noted.


Management Those suffering from TEN treated in the hospital, and if the

cause of the problem is drug related then the drug should be discontinued. The patient was given intravenous fluids to replace lost fluids, and the remaining skin to re-grow itself. However, the chances of survival can vary depending on the extent of damage and the degree of infection experienced by the patient.

500mg/kg dose IVIG with a single dose given per infusion for 6-8 hours is very effective for TEN.


Those who were taking medications that can cause TEN were advised to alert and able to identify the warning signs associated with this disease. Toxic epidermal necrolysis is a potentially deadly.


Prof DR Dr Ariyanto Harsono SpA(K) 33Thank You

Steven johnson syndrome & ten

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