Steven Johnson Syndrome – A Triad of Mucosal Lesions, Cutaneous Involvement and History of Recent Drug Exposure
Steven Johnson Syndrome is relative a rare but yet an acute life-threatening condition. A middle aged lady presented to us in an emergency condition with high grade fever (104oF), oral mucosal ulcers, purulent discharge from both eyes, photophobia, generalized skin rashes, flank bruises, dyspnea, severe odynophagia along with dysphagia and blisters on both hands and feet. Because of extensive oral thrush, mouth ulcers and purulent discharge from eyes, the patient was initially suspected to be a case of oral candidiasis and bacterial conjunctivitis. However, due to wide spread muco-cutaneous involvement and after obtaining detailed drug history and getting opinion from dermatology and ophthalmology department, a diagnosis of Steven Johnson Syndrome was established. The treatment was started accordingly and the patient responded dramatically to the treatment, got improved and was subsequently discharged.
2. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Archives of Dermatology. 1993 Jan 1;129(1):92-6.
3. Hebra F, Elfinger A, Heitzmann C. First descriptions in the International Atlas of rare skin diseases. Atlas der Hautkrankheiten 1969: 67-72
4. Lyell A. Toxic epidermal necrolysis (the scalded skin syndrome): a reappraisal. British Journal of Dermatology. 1979 Jan 1;100(1):69-86.
5. Rojeau JC, Stern RS. Severe cutaneous adverse reactions to drugs. N Engl J Med. 1994;331:1272-85.
6. Rzany B, Mockenhaupt M, Baur S, Schröder W, Stocker U, Mueller J, Holländer N, Bruppacher R, Schöpf E. Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990–1992): structure and results of a population-based registry. Journal of clinical epidemiology. 1996 Jul 31;49(7):769-73.
7. Schöpf E, Stühmer A, Rzany B, Victor N, Zentgraf R, Kapp JF. Toxic epidermal necrolysis and Stevens-Johnson syndrome: an epidemiologic study from West Germany. Archives of dermatology. 1991 Jun 1;127(6):839-42.
8. Chan HL, Stern RS, Arndt KA, Langlois J, Jick SS, Jick H, Walker AM. The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: a population-based study with particular reference to reactions caused by drugs among outpatients. Archives of Dermatology. 1990 Jan 1;126(1):43-7.
9. Chopra A, Drage LA, Hanson EM, Touchet NL. Stevens-Johnson syndrome after immunization with smallpox, anthrax, and tetanus vaccines. InMayo Clinic Proceedings 2004 Sep 30 (Vol. 79, No. 9, pp. 1193-1196). Elsevier.
10. Yetiv JZ, Bianchine JR, Owen Jr JA. Etiologic factors of the Stevens-Johnson syndrome. Southern medical journal. 1980 May;73(5):599-602.
11. Sendromu SJ. Stevens-Johnson Syndrome.
12. Roujeau JC. The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: a clinical classification. Journal of investigative dermatology. 1994 Jun 1;102(6).
13. Revuz J, Penso D, Roujeau JC, Guillaume JC, Payne CR, Wechsler J, Touraine R. Toxic epidermal necrolysis: clinical findings and prognosis factors in 87 patients. Archives of dermatology. 1987 Sep 1;123(9):1160-5.
14. Paul C, Wolkenstein P, Adle H, Wechsler J, Garchon HJ, Revuz J, Roujeau JC. Apoptosis as a mechanism of keratinocyte death in toxic epidermal necrolysis. British Journal of Dermatology. 1996 Apr 1;134(4):710-4.
15. Belafsky PC, Postma GN, Koufman JA, Bach KK. Stevens-Johnson syndrome with diffuse esophageal involvement.(Esophagoscopy Clinic). Ear, Nose and Throat Journal. 2002 Apr 1;81(4):220-1.
16. Agrawal A, Bramble MG, Shehade S, Dean J. Oesophageal stricturing secondary to adult Stevens-Johnson syndrome: similarities in presentation and management to corrosive injury. Endoscopy. 2003 May;35(5):454-7.
Bhoopat T, Bhoopat L. Sudden death in Stevens-Johnson syndrome: a case report. Forensic science international. 1994 Aug 10;67(3):197-203.
18. Leaute-Labreze C, Lamireau T, Chawki D, Maleville J, Taieb A. Diagnosis, classification, and management of erythema multiforme and Stevens–Johnson syndrome. Archives of disease in childhood. 2000 Oct 1;83(4):347-52.
19. Lane JM, Ruben FL, Neff JM, Millar JD. Complications of smallpox vaccination, 1968: national surveillance in the United States. New England Journal of Medicine. 1969 Nov 27;281(22):1201-8.
20. Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC. Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death?. Archives of Dermatology. 2000 Mar 1;136(3):323-7.
21. Esterly NB. Corticosteroids for erythema multiforme. Pediatr Dermatol. 1989;6:229-50.