STEVENS-JOHNSON SYNDROME

Definition:

 SJS is a generalized hypersensitivity reaction, usually to a drug, in which

the skin and mucus membrane lesions are early manifestation
 It may progress to its more severe form, toxic epidermal necrolysis
which has a high morbidity and up to 40% mortality.

Causes:

 Often unknown
 The most common causes include medications and infections
 Damage to the skin is thought to be mediated by cytotoxic T
lymphocytes and mononuclear cells
 Medications - especially sulfonamides, penicillins, anticonvulsants,
salicylates, NSAIDs, methazolamide, carvedilol (3,4) oxicams, and
allopurinol
 Vaccines - diphtheria/typhoid, bacillus Calmette Guerin (BCG), oral
polio vaccine (OPV)
 Infections: Mycoplasma pneumonia and herpes simplex are well-
recognized causes of erythema multiforme and SJS

Risk factors:

 Patients with HIV infection appear to be predisposed to developing SJS

in response to their medications
 Previous history of SJS
 Male sex

Signs and symptoms:

 There is usually a preceding illness for which medication was given

 Sudden onset with rapid progressive pleomorphic rash which includes
petechiae, vesicles, bullae
 The condition is classified as SJS if epidermal detachment affects less
than 10% of the skin, as toxic epidermal necrolysis (TEN) if epidermal
detachment exceeds 30%, or if it exceeds 10% in the absence of discrete
skin lesions.
 Cases with discrete skin lesions and between 10% and 30% epidermal
detachment are in the overlap between SJS and TEN
 Vesicles and ulcers on the mucous membranes, especially of the mouth
and throat
 Burning sensation of the skin and sometimes of the mucous membranes
 Usually no pruritus
 Fever 39-40°C (102-104°F)
 Headache
 Malaise
 Arthralgias
 Epistaxis
 Crusted nares
 Conjunctivitis
 Corneal ulcerations
 Erosive vulvovaginitis or balanitis
 Cough productive of thick purulent sputum
 Tachypnea/respiratory distress
 Albuminuria/hematuria
 Arrhythmias
 Pericarditis
 Congestive heart failure
 Mental status changes
 Electrolyte disturbance
 Seizures
 Coma
 Sepsis

Differential diagnosis:

 Exfoliative dermatitis
 Staphylococcal scalded skin syndrome
 Acute generalized exanthematic pustulosis
 Pemphigus (paraneoplastic) [Pemphigus vulgaris]
 Generalized fixed drug eruption
 Erythema multiforme major
 Burns
 Pressure blisters (coma, barbiturates)
 Bullous pemphigoid (a chronic bullous eruption most commonly
presenting in elderly people)
 Epidermolysis bullosa

Investigations:

Laboratory

 Electrolytes
 Liver enzymes
 Complete blood count
 Urinalysis
 Erythrocyte sedimentation rate (ESR)

Imaging/special tests

 Chest radiography if pneumonia is a consideration

 Skin biopsy of lesions and mucous membranes demonstrates necrosis of
the entire epidermal layer

Treatment

General measures:

 Withdrawal of any suspected medication, and treatment of any

underlying disease
 Meticulous care of damaged skin
 Reverse isolation when epidermal loss is extensive
 Maintenance of fluid, electrolyte and protein balance
 Adequate calorie intake, parenteral nutrition if necessary
 Oral hygiene with mouthwashes of warm saline, or solution of
diphenhydramine, lidocaine, and kaolin suspension
 Ophthalmological consultation and monitoring for corneal damage

Medications

 Administration of steroids is controversial.

 If there is no clear benefit within a few days, they should be withdrawn
 Experimental treatments that appear to have been useful include
recombinant granulocyte colony stimulating factor, plasmapheresis,
cyclophosphamide, cyclosporine, and, in HIV positive patients, IV
immune globulin.

Surgical measures:

 Sterile debridement (Excision of devitalized tissue and foreign matter

from a wound) of areas of extensive epidermal loss
 Application of biosynthetic dressings such as Biobrane to denuded areas
(13 )
 Long term damage to the vulva or vagina or to the cornea or may need
surgical repair.