Lyell's Syndrome

Lyell's syndrome, or toxic epidermal necrolysis or scalded skin syndrome, is a serious skin condition that usually develops in response to certain medications. This is the most severe variant of erythema exsudativum multiforme. It is characterized by the formation of blisters and significant linear peeling of large areas of skin (affects more than 30% of the body surface), mucous membranes are also affected. It is a life-threatening drug toxoallergic reaction. The incidence of Lyell's syndrome is rare, mortality is high.

. Not to be confused with Staphylococcal Scald Skin Syndrome (SSSS), which causes Staphylococcus aureus exfoliatin .

Etiology

 * It is formed after phenylbutazone, sulfonamides, ATB or barbiturates, and current infections also contribute to its formation.

Pathogenesis
The pathogenesis is still quite unclear. It is based on a cytotoxic cellular immunopathological response that is directed against antigens in the epidermis. TNF-α production and keratinocyte necrosis occur.

Clinical picture

 * In the prodromal stage of pharyngitis or otitis, then sowing of scarlatiform rash in the center of the face, perigenitally and perianally, finally blisters are sown, which turn into bulges, their coverage cracks and erosions with skin peeling occur.
 * It's usually accompanied by a general alteration of the patient's condition; disorders of consciousness, glomerulonephritis, pneumonia and hepatitis often occur.
 * positive Nikolsky symptom - pressure on the skin creates erosion.

Therapy

 * severe conditions can be treated with high doses of corticosteroids, i.v. immunoglobulins
 * larger affected areas must be treated as burns and fluid and ion balance maintained

Related articles

 * Stevensův-Johnsonův syndrom
 * Erythema multiforme

Reference

 * 1) BENEŠ, Jiří, et al. Infectious medicine. 1st edition. Prague: Galén, 2009. 651 pp.  ISBN 978-80-7262-644-1.
 * 2) BENEŠ, Jiří. Study materials  [online]. [feeling. 2010]. < http://jirben.wz.cz >.
 * 3) HAVLIK, Jiri, et al. Infectious diseases. 2nd edition. Prague: Avicenum, 1990. 393 pp.  ISBN 80-201-0062-8.
 * 4) BENEŠ, Jiří, et al. Infectious medicine. 1st edition. Prague: Galén, 2009. 651 pp.  ISBN 978-80-7262-644-1.
 * 5) ŠTORK, Jiří, et al. Dermatovenerology. 2nd edition. Prague: Galén, 2013. 502 pp.  ISBN 978-80-7262-898-8.