ERYTHEMA MULTIFORME, STEVENS JOHNSON SYNDROME, TOXIC EPIDERMAL NECROLYSIS

Erythema multiforme

An acute, self-limiting and commonly recurrent inflammatory skin eruption with variable involvement of the mucous membranes and without systemic symptoms. Symmetrically distributed crops of target lesions (dark centre, an inner, pale ring surrounded by an outer red ring) often involving palms and soles are characteristic. This condition is usually due to an infection, commonly herpes simplex or mycoplasma.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

An acute, systemic condition with vesico-bullous lesions involving the skin and mucous membranes. Non-specific prodromal symptoms, often mistaken as an upper respiratory tract infection, may occur before skin lesions are apparent.

Cutaneous lesions may start as a red morbilliform rash, progressing to purple skin necrosis and blisters which rupture, leaving large areas of denuded skin. The lesions exhibit a positive Nikolsky’s sign. Mucous membrane erosions are common and internal organ involvement may be present.

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This condition is usually due to medication, e.g. sulphonamides, antiretrovirals (nevirapine), anti-epileptics (phenytoin, phenobarbitone, carbamazepine, lamotrigine).

Systemic involvement with multi-organ dysfunction is common.

Complications include:

»     dehydration,

»     sepsis, and

»     adhesions and scarring.

Stop all medicines, including complementary, alternative, hormonal contraceptives and self medication.

GENERAL MEASURES

Principles of management

The foundation of management is supportive, good nursing and the prevention of dehydration and sepsis.

Stop all medicines.

Patients usually require care in a high or intensive care unit with dedicated nursing.

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Monitoring

Monitor vital organ function.  

Examine daily for infection and swab infected lesions. Do blood cultures if septicaemic.

Dressings

Skin hygiene; daily cleansing and bland, non-adherent dressings as needed.

Do not use silver sulfadiazine if condition is thought to be due to cotrimoxazole or other sulphonamide.

Mucous membranes:  Regular supervised oral, genital and eye care to prevent adhesions and scarring.

Two-hourly mouth washes with bland mouth wash, e.g. glycothymol. Examine daily for ocular lesions and treat 2-hourly with eye care and lubricants and break down adhesions.

Treat genitalia 6 hourly with Sitz baths and encourage movement of opposing eroded surfaces to prevent adhesions.

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Fluids: Oral rehydration is preferred but intravenous fluid therapy may be required in significant dehydration. Encourage oral fluids to prevent pharyngeal adhesions. Provide soft, lukewarm food or nasogastric feeds if unable to eat.

Note:

All patients should wear a notification bracelet/necklace.

MEDICINE TREATMENT

Corticosteroids

The practice of using systemic corticosteroids is not supported by evidence and is therefore not recommended.

Antibiotic therapy

Systemic antibiotics may be indicated, depending on results of appropriate cultures.

Analgesia

Appropriate and adequate analgesia for the pain associated with dressing changes, given at least half an hour before dressing change.

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