18.104.22.168 Stevens-Johnson Syndrome
Grading & Level of Importance: C
Rare disease 1-2 per 1 million. Higher risk in HIV/AIDS.
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.
Skin and mucosa.
Part of the Erythema multiforme –SJS/TEN complex as a clinical cascade.
Laboratory & other workups
Check electrolytes, kidney and liver parameters, cardiac function.
Cytotoxic T-lymphocytes attacking the epidermal keratinocytes, but also mucous epithelia. Apoptosis and necrosis of cells. Disruption of epidermal / dermal connection. Bulla formation.
Depends on age, comorbidity, body function status, earliest time of intervention. Electrolytes, bicarbonate and liver and kidney parameter.
Mortality 1 – 5 %. Scarring of blepharon, ectropium, phimosis, vaginal synechia.
Typical clinical features and histology. SCORTEN score recommended.
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).
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