Causes of Toxic Epidermal Necrolysis

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Drug reaction, skin care, skin loss.

Cooper KL. 2012 Aug;32(4):52-9.

Abstract

Stevens-Johnson syndrome is a rare, potentially fatal drug reaction that causes necrosis of epidermal cells. Early recognition of the syndrome is essential to prevent complications. This article discusses identification, complications, and treatment of Stevens-Johnson syndrome.

PMID:
22855079
[PubMed - in process]

Toxic epidermal necrolysis (also known as "Lyell's syndrome") is a rare, life-threatening dermatological condition that is usually induced by a reaction to medications. It is characterized by the detachment of the top layer of skin (the epidermis) from the lower layers of the skin (the dermis) all over the body.

There is broad agreement in medical literature that TEN can be considered a more severe form of Stevens–Johnson syndrome, and debate whether it falls on a spectrum of disease that includes erythema multiforme

Some authors consider that there is an overlap between the two syndromes (usually between 10% and 30% of skin detachment). The incidence is between 0.4 and 1.3 cases per million each year.

Signs and symptoms TEN affects many parts of the body, but it most severely affects the mucous membranes,
such as the mouth, eyes, and vagina.

The severe findings of TEN are often preceded by 1 to 2 weeks of fever. These symptoms may mimic those of a common upper respiratory tract infection. When the rash appears it may be over large and varied parts of the body, and it is usually warm and appears red. The dermal layer fills with fluid being deposited there by the body's immune system, usually as a result of a negative reaction to an antibiotic. The skin then begins to sag from the body and can be peeled off in great swaths.

The mouth becomes blistered and eroded, making eating difficult and sometimes necessitating feeding through a nasogastric tube through the nose or a gastric tube directly into the stomach. The eyes are affected, becoming swollen, crusted, and ulcerated and blindness may occur. [edit] Pathology File:Confluent epidermal necrosis — very high

Micrograph showing full thickness epidermal necrosis with a basket weave-like stratum corneum and separation of the dermis and epidermis.

Skin biopsy. H&E stain. TEN, like Steven-Johnson syndrome and erythema multiforme, are characterized by confluent epidermal necrosis with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum.


Toxic epidermal Necrolysis and Stevens-Johnson Syndrome personal.

Toxic epidermal Necrolysis images and Stevens-johnson syndrome deadly adverse drug reaction.

Toxic epidermal necrolysis video.


The management of toxic epidermal necrolysis
Suran L Fernando*

ABSTRACT

The mortality rate of Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high; approximately 5% for SJS and 25% for TEN. It is therefore vital for the treating physician to recognise SJS and TEN promptly through the identification of these diseases' characteristic clinical features so that the offending drug is promptly withdrawn, supportive therapy is administered and adjunctive therapies are considered.

Supportive therapy addressing the manifestations and complications of acute skin failure include monitoring the fluid – electrolyte balance and providing enteral or parenteral nutrition, wound care and treatment of sepsis. In addition, supportive care of the affected mucosal surfaces is required through the use of aggressive ocular lubrication, topical corticosteroids, hygienic mouthwashes and oral anaesthetics, together with monitoring for urinary retention....

The role of plasmapheresis, anti-tumour necrosis factor (TNF) biologics and N-acetylcysteine is promising but further studies are required to elucidate their benefit. Preventative strategies such as pharmacogenetic screening needs to be strongly considered, with the provision of cost-effective assays with a rapid turn-around time.

 


SJS and TEN: A Quick Review

 • Sometimes starts with fever, stinging eyes, and painful swallowing.

Patients experience skin tenderness, erythema, epidermal necrosis, and desquamation.

 • Dusky (blue to black) erythematous macules progress to flaccid blisters.

• Two or more mucous membranes are usually involved with buccal, genital, and ocular mucosa erythema and erosions. • Massive fluid loss and electrolyte imbalance follows epidermal detachment. These conditions are potentially life-threatening because of their multisystem involvement and skin-barrier breakdown. Epithelial loss results in vulnerability to bacterial and fungal infections and predisposes patients to septicemia and severe fluid loss. Mortality ranges from 5% in SJS to 30% in TEN.

 • Survivors may suffer from mucous membrane strictures. • Severe ophthalmic involvement may lead to permanent scarring and blindness.