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IVIG boosts survival in toxic epidermal necrolysis: reduction in mortality.

LAS VEGAS -- Eighteen of 19 consecutive toxic epidermal necrolysis patients survived after receiving intravenous immunoglobulin at the University of Miami, reported Dr. Francisco Kerdel at a dermatology seminar sponsored by the Skin Disease Education Foundation.

The mortality rate for toxic epidermal necrolysis (TEN) generally hovers around 30%-35%, however, some recent reports have suggested that intravenous immunoglobulin (IVIG) might improve the odds for patients with the rare, rapidly progressive response to sulfonamides, anticonvulsive agents, and other medications (Arch. Dermatol. 139[1]:85-86, 2003).

Use of IVIG is thought to inhibit FasFasL ligand interactions that mediate apoptosis of keratinocytes during rapid exfoliation that characterizes the mucocutaneous disorder.

A recent French study, though, reported a higher-than-expected mortality among patients receiving MG, leaving the issue unresolved (Arch. Dermatol. 139[1]:33-36, 2003).

A severity-of-illness score, SCORTEN, which predicts outcome based on seven independent risk factors for death, suggest ed that six of the University of Miami patients would have been expected to die of TEN due to their age, initial presentation, laboratory values, and other factors.

Yet only 1 patient of 19 died, said Dr. Kerdel, professor of dermatology and cutaneous surgery at the university.

That patient received a lower dosage--0.4 g/kg per day--than the others, who all received 1 g/kg per day of IVIG for 4 days after admission for TEN.

"In Miami, if you get gamma globulin, you're 83.5% less likely to die," remarked Dr. Kerdel.

Administration of IVIG is not by any means the only key to survival in the Miami TEN program, Dr. Kerdel emphasized.

Patients are rapidly admitted to the intensive care unit, not to a burn unit. There, they receive multifaceted care for fluid loss, infection, impaired thermal regulation, reduced energy stores, and pain.

"You have to recognize that this condition represents an acute skin failure," he stressed.

Steroids are discontinued, since no good evidence supports their use. A nasogastric tube is placed for delivery of fluid and nutrition. The patient's temperature is controlled and electrolyte balance restored. Cultures are obtained, with a special eye for staphylococcus early in the course of treatment and Pseudomonas later on.

Dr. Kerdel cautioned against fixed fluid resuscitation, standard in burn units, in favor of aiming at a steady urine output of 40-60 mL/hr. He favors colloid fluid replacement--specifically, 25% albumin delivered in a bolus.

Antacids, anticoagulants, and analgesics are administered as needed, and special attention is paid to eye care, since ocular involvement is common in TEN and can be severe.

Patients are encased in a special nonadherent, three-layer dressing infused with 0.5% silver nitrate to protect the body from rapid skin loss and to restore fluids. Unlike traditional gauze, exudate does not stick to the dressing when it is removed, and after as soon as 3 days, newly formed epithelium can be seen, said Dr. Kerdel.

Not enough evidence exists for Dr. Kerdel to recommend hyperbaric oxygen, cyclosporine, or removal of blistered skin in TEN patients.

Some experts have advocated placing TEN patients on air fluid beds, but he believes the increased evaporation may complicate the fluid balance in these patients.

The Skin Disease Education Foundation and this newspaper are wholly owned subsidiaries of Elsevier.


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Title Annotation:Clinical Rounds; intravenous immunoglobulin
Author:Bates, Betsy
Publication:Family Practice News
Geographic Code:1USA
Date:Feb 1, 2004
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