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Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
ISSN: 0378-6323 EISSN: 0973-3922
Vol. 75, Num. 6, 2009, pp. 613-614

Indian Journal of Dermatology, Venereology and Leprology, Vol. 75, No. 6, November-December, 2009, pp. 613-614

Letter to the Editor

Outcome of Stevens Johnson syndrome and toxic epidermal necrolysis treated with corticosteroids

Department of Dermatology, B. P. Koirala Institute of Health Science, Dharan, Nepal

Correspondence Address: Dr. Arpana Rijal, Department of Dermatology and Venereology, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.
arpanarijal@yahoo.co.uk

Code Number: dv09210

PMID: 19915248

DOI: 10.4103/0378-6323.57729

Sir,

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are drug-induced or idiopathic reaction patterns characterized by skin tenderness, along with erythema of the skin, followed by extensive cutaneous and mucosal sloughing. They are life-threatening due to multisystem involvement and mortality ranges from 25 - 70%. [1] SJS includes cases with less than 10% epidermal detachment, mucosal lesions, and widespread purpuric lesions; SJS / TEN overlap when the epidermal detachment is between 10 and 30%; mucosal lesions, widespread purpuric lesions, and TEN when the epidermal detachment is more than 30%, and mucosal lesions and widespread purpuric lesions are present. [2] Early intervention with corticosteroids controls inflammation, [2] as corticosteroids are potent agents that target several intracellular processes, to modify almost all components of inflammatory and immune responses, hence, some favor early use of corticosteroids. Some studies suggest that systemic steroids adversely affect the outcome by increasing the risk of septicemia and gastrointestinal bleeding. [3],[4],[5],[6]

A retrospective analysis of the records of patient's admitted in the dermatology ward with SJS,SJS-TEN overlap, and TEN, between 1997 and 2005, was performed. A detailed study of case records regarding clinical presentation, investigations, treatments, treatment outcome, and provoking factor was done.

The total number of patients admitted with SJS was 10 (41.6%), with SJS-TEN overlap was eight (33.3), and with TEN was six (23%). Mean age of the patients was 26.4 years. The mean percentage of body surface area involved was 35.5%. The patients reported to the hospital within 1.9 days of appearance of the lesions. Prodromal signs were seen in all the patients.

The drugs implicated in the decreasing order of frequency were phenytoin 8 (33.3%) carbamazapine 5 (20.6%), sulfonamides 5 (20.6%), amoxicillin 3 (12.5%), ibuprofen 2 (8.33%), and ciprofloxacin 1 (4.16%). Viral infection was seen in one case [Table - 1].

The offending drug was stopped immediately. The patients were bathed daily and paraffin gauze was applied over the raw body surface area. Antibiotics, ceftriaxone, and gentamycin were given prophylactically. Twenty-two patients were started on oral or intravenous (IV) corticosteroids with doses ranging from 1 to 3 mg / kg / body weight. Oral pednisolone was given in patients who could take it orally. Dexamethasone was given IV. Corticosteroids were tapered according to the response seen. The patients, received corticosteroids for 14 - 30 days (mean 15 days). Two patients did not receive corticosteroids as one had sepsis and in the other the etiology was of viral origin, the patient had chicken pox. A Tzanck smear was done, which showed multiple multinucleated giant cells, and serum IgM was raised above normal. Strict monitoring of the patients' vitals was done daily. Sepsis screening was done at baseline and twice weekly. All the patients recovered. The average period of stay in hospital was 19.4 days (range 7 - 41 days). All patients were kept in isolation.

The principle of symptomatic treatment are the same as that for burns and include fluid replacement, nutritional support, and antimicrobial therapy. [3] Early reporting by our patients, within 1.9 days of appearance of lesions, and the early use of corticosteroids may have favored a better prognosis. Patterson [7] reported the effectiveness of early steroid administration in the Stevens-Johnson syndrome in 41 cases.

Lesser total body surface area involvement showed a better survival rate as seen in other studies. [8] Most of our patients, 75% (18 cases), had 30% or less than 30% of body surface area involvement. Younger patients had better prognosis as seen from other studies. [9],[10],[11] The majority of our patients were between 18 and 30 years. From our analysis we have seen that steroids, when given early, preferably within 72 hours, in younger patients, with lesser body surface area involvement, without any comorbidity, and under very strict supervision, preferably in well-equipped centers, prove beneficial to the patients with SJS / TEN.

References

1.Prendiville JS, Hebert AA, Greenwald MJ, Esterly NB. Management of Stevens - Johnson syndrome and TEN. J Pediatr 1989;115:881-7.  Back to cited text no. 1    
2.Chave TA, Mortimer NJ, Sladden MJ, Hall AP, Hutchinson PE. Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005;153:241-53.  Back to cited text no. 2    
3.Heimbach DM, Engrav LH, Marvin JA, Harnar TJ, Grube BJ. Toxic epidermal necrolysis: a step forward in treatment: JAMA 1987;257:2171-5.  Back to cited text no. 3    
4.Rzany B, Schmitt H, Schöpf E. Toxic epidermal necrolysis in patients receiving glucocorticosteroids. Acta Derm Venereol 1991;71:171-2.  Back to cited text no. 4    
5.Hansbrough JF, Muller P, Noordenbos J, Dore C. A 10-year experience with toxic epidermal necrolysis. J Burn Care Rehabil 2001;22:97-8.  Back to cited text no. 5    
6.Smoot EC 3rd. Treatment issues in the care of patients with toxic epidermal necrolysis: Burns 1999;25:439-42.  Back to cited text no. 6    
7.Patterson R, Miller M, Kaplan M, Doan T, Brown J, Detjen P, et al. Effectiveness of early therapy with corticosteroids in Stevens-Johnson syndrome: experience with 41 cases and a hypothesis regarding pathogenesis. Ann Allergy 1994;73:27-34.  Back to cited text no. 7    
8.Engelhardt SL, Schurr MJ, Helgerson RB. Toxic Epidermal Necrolysis: an analysis of referral pattern and steroid usage. J Burn Care Rehabil 1997;18:520­-4.  Back to cited text no. 8    
9.Criton S, Devi K, Sridevi PK, Asokan PU. Toxic Epidermal necrolysis - a retrospective study. Int J Dermatol 1997;36:923-5.  Back to cited text no. 9    
10.Tripathi A, Ditto AM, Grammer LC, Greenberger PA, McGrath KG, Zeiss CR, et al. Corticosteroid therapy in an additional 13 cases of Stevens - Johnson syndrome: a total series of 67 cases. Allergy Asthma Proc 2000;21:101-5.  Back to cited text no. 10    
11.Schulz JT, Sheridan RL, Ryan CM, MacKool B, Tompkins RG. A ten year experience with toxic epidermal necrolysis. J Burn Care Rehabil 2000;21:199-204.  Back to cited text no. 11    

Copyright 2009 - Indian Journal of Dermatology, Venereology and Leprology


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