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   Toxic Epidermal Necrolysis

Stevens Johnson Syndrome and Toxic Epidermal Necrolysis are life-threatening conditions.

Although infrequent, these conditions kill or severely disable previously healthy people. (Roujeau, 1995) 

Both diseases are characterized by rapidly expanding rashes, often with atypical (flat, irregular) target lesion, and involvement of the mucous membranes (mouth, eyes, and genital areas)

Toxic Epidermal Necrolysis

Indian J Dermatol Venereol Leprol. 2005 Nov-Dec;71(6):398-400. Related Articles, Links

Efficacy of low dose intravenous immunoglobulins in children with toxic epidermal necrolysis: An open uncontrolled study.

Mangla K, Rastogi S, Goyal P, Solanki RB, Rawal RC.

Department of Dermatology, V. S. Hospital, Ahmedabad, India. ranjanmb@yahoo.com.

BACKGROUND: High dose intravenous immunoglobulins (IVIG) have emerged as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies support the view that IVIG can block the fas-fas ligand mediated apoptosis in TEN. METHODS: Ten pediatric patients of TEN were treated with IVIG (0.05 - 0.1 gm/kg/day) along with antibiotics and supportive care. RESULTS: Patients with 67% of mean body surface area of involvement showed an average of 2.1 days for arrest of progression of lesions and 8.1 days for complete reepithelization. There was no mortality. CONCLUSIONS: Low dose IVIG appears to be a safe and effective treatment for TEN in children. Randomized trials are needed to further evaluate the efficacy of IVIG and compare it with other therapeutic modalities.

PMID: 16394480 [PubMed - in process]
 

Toxic epidermal necrolysis

Indian J Dermatol Venereol Leprol. 2005 Jan-Feb;71(1):23-5. Related Articles, Links

A study of mortality in dermatology.

Nair PS, Moorthy PK, Yogiragan K.

Departments of Dermatology and Venereology, Medical College Hospital, Trivandrum - 695 011, India. sysantosh@yahoo.com.

BACKGROUND AND AIMS: Certain dermatological conditions are life-threatening and can cause mortality. The aim of this study is to find out the dermatological diseases leading to death in our indoor patients. METHODS: A record-based retrospective descriptive study of dermatology cases who died during the period of 1995 to 2001. RESULTS: The total number of cases analyzed was thirty-seven. There were 24 males and 13 females. The maximum number of deaths occurred in the age group 61-70. Vesiculobullous disorders were the commonest cause of death, found in 18 cases (48.6%), followed by drug reactions in 5 (13.5%), malignancies in 5 (13.5%) and collagen vascular disease in 2 cases ((5.40%). Pemphigus was the commonest fatal vesiculobullous disorder (13 cases - 35.13%), and toxic epidermal necrolysis the commonest drug reaction (3 cases - 8.10%). CONCLUSION: Area of skin involvement, electrolyte imbalance and septicemia were important factors leading to death in pemphigus and toxic epidermal necrolysis. We advocate that such patients should be managed in burns unit or ICU units.

PMID: 16394356 [PubMed - in process]

 
J Invest Dermatol. 2005 Dec 22; [Epub ahead of print] Related Articles, Links

Performance of the SCORTEN During the First Five Days of Hospitalization to Predict the Prognosis of Epidermal Necrolysis.

Guegan S, Bastuji-Garin S, Poszepczynska-Guigne E, Roujeau JC, Revuz J.

1Department of Dermatology, Henri-Mondor Hospital (AP-HP), University Paris XII, Creteil, France.

The SCORTEN, calculated within 24 hours of admission, is a severity-of-illness score validated for toxic epidermal necrolysis and Stevens-Johnson syndrome. Our purpose was to assess the performance of successive SCORTEN during the first 5 days of hospitalization and to determine the influence of admission delay. Charts of 144 patients aged 46.8 years (+/-19.7), admitted to our department (1993-2003) with Stevens-Johnson syndrome or toxic epidermal necrolysis, were reviewed. Successive SCORTEN were compared between deceased patients (n=28, 19.4%) and survivors (n=116). The performance of the score (calibration, discrimination) was assessed on days 1-5. All seven SCORTEN variables, on days 1-5, were associated with a higher mortality rate. The SCORTEN rose slightly during hospitalization, with a significant difference between days 1 and 4 (<0.05). Performance of the SCORTEN was good on each day, but slightly better on day 3. The areas under the receiver-operating characteristic curves were above 80%. The admission delay did not differ between deceased patients and survivors. Delay-adjusted SCORTEN was close to the crude SCORTEN. The SCORTEN performance during the first 5 days of hospitalization was excellent, and at its best on day 3. We recommend to compute again the SCORTEN on day 3. The admission delay did not influence prognosis or SCORTEN.Journal of Investigative Dermatology advance online publication, 22 December 2005; doi:10.1038/sj.jid.5700068.

PMID: 16374461 [PubMed - as supplied by publisher]

Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme

S. Bastuji-Garin, B. Rzany, R. S. Stern, N. H. Shear, L. Naldi and J. C. Roujeau
Department of Dermatology, Henri-Mondor Hospital, University of Paris XII, Creteil, France.

BACKGROUND AND DESIGN. To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. RESULTS. The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. CONCLUSION. This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.