Gülhan YAĞMUR, Murat Nihat ARSLAN, Ferah KARAYEL ANIK, Hüseyin EŞ

TOKSİK EPİDERMAL NEKROLİZİS BENZERİ BÜLLÖZ CİLT REAKSİYONU: BİR OTOPSİ OLGUSU

Toksik epidermal nekrolizis epi- dermisin dermisten ayrılmasıile karakterize bir durumdur.Deride maküler lezyonların üze- rinde vezikül ile bül oluşumu iz- lenir ve bunlar kolayca yırtılarakyerlerinde erode alanlar kalır.Patogenezi tam olarak bilinme- mekle birlikte toksik epidermalnekrolizis genellikle ilaçlaradaha az sıklıkla da hepatit, mi- koplazma, varisella, herpes,Escherichia coli septiseminebağlı gelişir. Antibiyotikler, an- tikonvülzan ve nonsteroidal an- tiinflamatuar ilaçlar etiyolojideen sık karşılaşılan ilaçlardır.Mortalitesi yüksek bir tablo olupçeşitli yayınlarda %20-60 ara- sında olduğu bildirilmiştir. Ensık ölüm nedeni sepsis ve bunabağlı olarak gelişen akciğer tu- tulumudur. Ölüme neden olansepsisten sorumlu mikrobiyalajanlar genellikle Enterococcusspp, Acinetobacter spp, Staph- ylococcus aureus, Pseudoma- nas aurigonasadır. 40 yaşında erkek olgu, bilincikapalı bir şekilde 112 ambu- lansı ile hastaneye getirilmişve gelişen kardiyak arrest ne- deniyle yoğun bakım servisindetakip edilmeye başlanmıştır. 1haftalık takip sonrasında ölümünedeniyle kesin ölüm sebebinintespiti için otopsiye gönderil- miştir. Olgunun dış muayenesinde sak- ral ve gluteal bölgede bası yara- ları, göğüs ve sırt bölgeleri hariçtüm vücutta yer yer epidermalbüller ve büllerin patlamasınabağlı tabanı hiperemik yer yermor kırmızı renk değişikliği olanepidermal soyulmalar izlenmiş- tir. İç muayenede göğüs boş- luklarında ve karında kirli sarırenkte mayi boşaltıldı. Trakea,özefagus alt kısmı ve omentumenfekte görünümde izlenmiştir.Histopatolojik incelemede ak- ciğerde taze lobuler pnömoni,bronşit, ince ve kalın bağırsak- larda şiddetli aktif enterit, kolitgörülmüştür. Yapılan toksikolo- jik incelemede kanda 78,4ng/mlPentobarbital, 169ng/ml Thio- pental, Pheniramine, Ranitidine,Metoclopromid tespit edilmiştir.Mikrobiyolojik örneklerde kan- da ve beyin omurilik sıvısındaE. coli, Enterococcus faecium,akciğerde E. coli, Candida al- bicans, dalak ve karaciğerde E.coli, batın sıvısında E. coli, Pro- teus spp, bül sıvısında E. coli,Enterococcus faecium ürediğigörülmüş olup prokalsitonindüzeyi 2,26ng/ml bulunmuştur.Olgunun ölüm sebebi yaygın sis- temik enfeksiyon olarak düşü- nülmüştür.

TOXIC EPIDERMAL NECROLYSIS LIKE BULLOUS SKIN REACTION: AN AUTOPSY CASE

Toxic epidermal necrolysis (TEN)is characterized by detachment ofepidermis from underlying der- mis. Vesicular and bullous lesionsarise on macules. These lesionsrupture easily and eroding lesionsremain. Pathogenesis of TEN isnot fully clear yet but in generalsome medications, hepatitis, sep- ticemias of Mycoplasma, Varicella,Herpes, Escherichia coli infectionsare suspected. Antibiotics, anti- convulsant drugs or nonsteroidalanti-inflammatory drugs are mostcommonly involved drugs. In somepublications mortality rate report- ed as between 20 and 60 per cent.Most common cause of death issepsis and subsequent pulmonaryinvolvement in TEN. Enterococcusspp, Acinetobacter spp, Staphylo- coccus aureus, Pseudomanas au- rigonasa are most common causesof sepsis in those cases. A forty year-old man, transportedto the hospital in unconscious statewithout any medical or judicial an- tecedent and followed in intensivecare unit because of cardiac arrest.After one week follow-up patientdied and referred to the Council ofForensic MedicineThere were widespread (exceptchest and back) epidermal bullouslesions and concomitant rupturedbullae with hyperemic and purple- colored underlying tissue. During autopsy there were yellow effusionsin chest and abdominal cavity.Lower segments of trachea andesophagus and greater omen- tum had infective appearance. Onthe histopathological examinationof lungs lobular pneumonia andbronchitis were detected. Severeactive enteritis and colitis weredetected on small intestine andbowel specimens. On the toxicolog- ical screening of blood, 78.4ng/mlPentobarbital, 169ng/ml Thiopen- tal; Pheniramine, Ranitidine andMetoclopromide were detected. Onpostmortem microbiological studyof blood and cerebrospinal fluid E.coli, Enterococcus faecium; at thelung tissue E. coli, Candida albi- cans; on the splenic tissue E. coliwere colonized. On the microbio- logical study of abdominal effusionE. coli and Proteus spp were colo- nized and on bullae fluid E. coli andEnterococcus faecium were colo- nized. Serum Procalcitonin levelwas 2.26ng/ml. Cause of death de- termined as widespread systemicinfection.

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1. Yamane Y, Aihara M, Ikezawa Z. Analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis in Japan from 2000 to 2006. Allergol Int 2007;56(4):419-25. 2. Mockenhaupt M, Viboud C, Dunant A, Na- ldi L, Halevy S, Bouwes Bavinck JN, Sidoroff A, Schneck J, Roujeau JC, Flahault A. Ste- vensJohnson syndrome and toxic epider- mal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Der- matol 2007;128(1):35-44. 3. Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol 1956;68(11):355-61. 4. Lyell A. Toxic epidermal necrolysis (the scalded skin syndrome): A reappraisal. Br J Dermatol 1979;100(1):69-86. 5. Gravante G, Delogu D, Marianetti M, Trombetta M, Esposito G, Montone A. Toxic epidermal necrolysis and Steven John- son syndrome: 11-years experience and outcome. Eur Rev Med Pharmacol Sci 2007;11(2):119-27. 6. Tezer H, Devrim İ, Kara A, Cengiz AB, Seçmeer G. Adolesan kız hastada antibiyotik ilişkili toksik epidermal nekrolizis ve intra- venöz immünglobülin tedavisi. Cocuk Sag Hast Derg 2007;50(4):254-8. 7. Yalcin AD, Karakas AA, Soykan G, Gorc- zynski RM, Sezer C, Bisgin A, et al. A case of Toxic Epidermal Necrolysis with diverse etiologies: successful treatment with intra- venous immunoglobulin and pulse pred- nisolone and effects on sTRAIL and sCD200 levels. Clin Lab 2013;5(6):1-5. 8. Lee H-Y, Chung W-H. Toxic epidermal necrolysis: the year in review. Curr Opin Al- lergy Clin Immunol 2013;13(4):330-6. 9. Chave TA, Mortimer NJ, Sladden MJ, Hall AP, Hutchinson PE. Toxic epidermal necrol- ysis: current evidence, practical manage- ment and future directions. Br J Dermatol 2005;153(2):241-53. 10. Le Cleach L, Delaire S, Boumsell L, Bagot M, Bourgault-Villada I, Bensussan A, Roujeau JC. Blister fluid T lymphocytes dur- ing toxic epidermal necrolysis are functional cytotoxic cells which express human natural killer (NK) inhibitory receptors. Clin Exp Im- munol 2000;119(1):225-30. 11. Nassif A, Bensussan A, Dorothée G, Ma- mi-Chouaib F, Bachot N, Bagot M, Boum- sell L, Roujeau JC. Drug specific cytotoxic T-cells in the skin lesions of a patient with toxic epidermal necrolysis. J Invest Derma- tol 2002;118(4):728-33. 12. Cohen S, Billig A, Ad-El D. Ceftriaxone- induced toxic epidermal necrolysis mimick- ing burn injury: a case report. J Med Case Reports 2009;3(1):9323. 13. Roujeau J-C, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T, Auquier A, Bastuji- Garin S, Correia O, Locati F, Mockenhaupt M, Paoletti C, Shapiro S, Shear N, Schöpf E, Kaufman DW. Medication use and the risk of StevensJohnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;333(24):1600-8. 14. Plaut ME, Mirani M. Toxic epider- mal necrolysis due to e coli. JAMA 1972;219(12):1629-32. 15. Ducic I, Shalom A, Rising W, Nagamoto K, Munster AM. Outcome of patients with toxic epidermal necrolysis syndrome revisit- ed. Plast Reconstr Surg 2002;110(3):768-73. 16. Sekula P, Dunant A, Mockenhaupt M, Na- ldi L, Bouwes Bavinck JN, Halevy S, Kardaun S, Sidoroff A, Liss Y, Schumacher M, Rou- jeau JC. Comprehensive survival analysis of a cohort of patients with StevensJohnson Syndrome and Toxic Epidermal Necrolysis. J Invest Dermatol 2013;133(5):1197-204. 17. Güneş A, Yolbaş İ, Kelekçi S, Yel S, Şahin C, Ece A. Successful management of a pa- tient with toxic epidermal necrolysis by high dose intravenous immunoglobulin. J Clin Exp Invest 2013;4(4):503-505. 18. Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis 2010;5(1):39. 19. Das S, Roy AK, Biswas I. A six-month prospective study to find out the treatment outcome, prognosis and offending drugs in toxic epidermal necrolysis from an urban institution in Kolkata. Indian J Dermatol 2013;58(3):191-3. 20. Bachot N, Roujeau J-C. Differential di- agnosis of severe cutaneous drug eruptions. Am J Clin Dermatol 2003;4(8):561-72.