Stevens-Johnson Syndrome

ICD-11

EB13

Synonyms

SJS

Epidemiology

Rare disease 1-2 per 1 million. Higher risk in HIV/AIDS.

Definition

Part of a spectrum of skin and mucosal disease, which ranges from SJS, where 30% of the BS shows loss of the epidermis. Involvement of 10% to 30 % of the BS is referred to as SJS-TEN-overlap syndrome.

Aetiology & Pathogenesis

Many causes, most often medications, less often infections (viruses, mycoplasma). Hereditary disposition to severe cutaneous drug reactions. Chinese and Thai HLA-B*1502 related, in Europeans HLA-A*3101. In both groups HLA-B*5801 related to allopurinol.

Signs & Symptoms

Macular exanthem, usually on trunk, often becoming confluent, with formation of atypical target lesions. Mucosal involvement is mandatory and erosions present around and inside more than one mucosal orifice. Fever, conjunctivitis, throat pain, balanitis, later upper respiratory and gastrointestinal tract involved. Nikolski I sign positive.

Localisation

Skin and mucosa.

Classification

Part of the Erythema multiforme –SJS/TEN complex as a clinical cascade.

Laboratory & other workups

Check electrolytes, kidney and liver parameters, cardiac function.

Dermatopathology

Cytotoxic T-lymphocytes attacking the epidermal keratinocytes, but also mucous epithelia. Apoptosis and necrosis of cells. Disruption of epidermal / dermal connection. Bulla formation.

Course

Depends on age, comorbidity, body function status, earliest time of intervention. Electrolytes, bicarbonate and liver and kidney parameter.

Complications

Mortality 1 – 5 %. Scarring of blepharon, ectropium, phimosis, vaginal synechia.

Diagnosis

Typical clinical features and histology. SCORTEN score recommended.

Differential diagnosis

Disseminated herpes simplex infection (primary course), drug reaction, pemphigus vulgaris, DRESS.

Prevention & Therapy

Therapy has to be adapted to course of the development of body surface and mucosal involvement. Patient should be directly referred to a dermatologic center.
Decision on systemic corticosteroids, IV Immunoglobulins, TNF alpha blockers, and symptomatic topical measures will be done according to course. Often treated in a similar manner to TEN (see TEN).

1.1.3.4 Stevens-Johnson Syndrome