RAMB - Agosto 2016

tevens

-J

ohnson

syndrome

and

toxic

epidermal

necrolysis

review

ssoc

ras

2016; 62(5):468-473

471

good history, with emphasis on the use of drugs and the

occurrence of previous infections, and thorough physi-

cal examination are essential.

Drug provocation tests are contraindicated, since a

subsequent exposure to the agent could trigger a new se-

vere episode of SJS/TEN.

Complete blood count (CBC) may show unspecific

leukocytosis or even indicate superimposed secondary

bacterial infection. Cultures of blood, urine and skin may

reveal the agent of the underlying suspected infection.

Skin biopsy is an additional final examination and

reveals necrosis in all layers of the epidermis caused by

apoptosis of keratinocytes and epidermal detachment,

while the dermis displays minimum inflammatory chang-

(Figure 2).

Serum levels of tumor necrosis factor-alpha and sol-

uble Il-2, Il-6 and C-reactive protein receptors are typi-

cally elevated in patients with SJS, although none of

these serological tests are used routinely for diagnosis

in our midst.

ifferential

diagnosis

The most important differential diagnoses include dis-

orders involving the peeling of the skin, such as erythe-

ma multiforme major, herpes simplex virus (HSV)–asso-

ciated erythema multiforme, burns, widespread fixed drug

eruption, acute generalized exanthematous pustulosis,

erythroderma, bullous pemphigoid, linear IgA dermato-

sis, paraneoplastic pemphigus, lymphoma, angioimmu-

noblastic lymphadenopathy, viral rashes, secondary syph-

ilis, herpetic gingivostomatitis, staphylococcal scalded

skin syndrome, graft

versus

host disease and autoimmune

vasculitis.

1-3,13

reatment

Management of patients with SJS or TEN requires three

measures: removal of the offending drug, particularly

drugs known to be high-risk; supportive measures and

active interventions.

Early diagnosis of the disease, recognition of the caus-

al agent and the immediate withdrawal of the drug are

the most important actions, as the course of the disease

is often rapid and fatal.

Figuring out the offending drug may not be easy and,

in these cases, all drugs non-essential to the maintenance

of the patient’s life should be suspended.

upport

treatment

Patients should preferably be treated in burn units. The

first care should include supportive and symptomatic

measures: body temperature control, hydration and elec-

trolyte replacement, special attention to the airways, pre-

venting secondary infection, pain control, maintenance

of venous access distant from the affected areas, early oral

nutrition or parenteral nutrition, if necessary, and anti-

coagulation.

7,14

Skin lesions are treated according to the protocol for

patients with large burns. There is no consensus on top-

ical care. Topical antiseptics can be used, or just soap and

water, in quick baths.

The practice of debridement is controversial.

Prophylactic antibiotic therapy is not recommended

as it can induce resistance and because these drugs can

per se

be causative agents of SJS or TEN. Therefore, they

are given only in proven cases of infection, or when there

is sudden decrease/rise in temperature, poor general con-

dition, or positive skin cultures.

FIGURE 2

Pathological examination of the skin of a patient with Stevens-Johnson syndrome.