TOKSİK EPİDERMAL NEKROLİZİS BENZERİ BÜLLÖZ CİLT REAKSİYONU: BİR OTOPSİ OLGUSU

Öz

Toksik epidermal nekrolizis epidermisin dermisten ayrılması ile karakterize bir durumdur. Deride maküler lezyonların üzerinde vezikül ile bül oluşumu izlenir ve bunlar kolayca yırtılarak yerlerinde erode alanlar kalır. Patogenezi tam olarak bilinmemekle birlikte toksik epidermal nekrolizis genellikle ilaçlara daha az sıklıkla da hepatit, mikoplazma, varisella, herpes, Escherichia coli septisemine bağlı gelişir. Antibiyotikler, antikonvülzan ve nonsteroidal antiinflamatuar ilaçlar etiyolojide en sık karşılaşılan ilaçlardır. Mortalitesi yüksek bir tablo olup çeşitli yayınlarda %20-60 arasında olduğu bildirilmiştir. En sık ölüm nedeni sepsis ve buna bağlı olarak gelişen akciğer tutulumudur. Ölüme neden olan sepsisten sorumlu mikrobiyal ajanlar genellikle Enterococcus spp, Acinetobacter spp, Staphylococcus aureus, Pseudomanas aurigonasa’dır. 40 yaşında erkek olgu, bilinci kapalı bir şekilde 112 ambulansı ile hastaneye getirilmiş ve gelişen kardiyak arrest nedeniyle yoğun bakım servisinde takip edilmeye başlanmıştır. 1 haftalık takip sonrasında ölümü nedeniyle kesin ölüm sebebinin tespiti için otopsiye gönderilmiştir. Olgunun dış muayenesinde sakral ve gluteal bölgede bası yaraları, göğüs ve sırt bölgeleri hariç tüm vücutta yer yer epidermal büller ve büllerin patlamasına bağlı tabanı hiperemik yer yer mor kırmızı renk değişikliği olan epidermal soyulmalar izlenmiştir. İç muayenede göğüs boşluklarında ve karında kirli sarı renkte mayi boşaltıldı. Trakea, özefagus alt kısmı ve omentum enfekte görünümde izlenmiştir. Histopatolojik incelemede akciğerde taze lobuler pnömoni, bronşit, ince ve kalın bağırsaklarda şiddetli aktif enterit, kolit görülmüştür. Yapılan toksikolojik incelemede kanda 78,4ng/ml Pentobarbital, 169ng/ml Thiopental, Pheniramine, Ranitidine, Metoclopromid tespit edilmiştir. Mikrobiyolojik örneklerde kanda ve beyin omurilik sıvısında E. coli, Enterococcus faecium, akciğerde E. coli, Candida albicans, dalak ve karaciğerde E. coli, batın sıvısında E. coli, Proteus spp, bül sıvısında E. coli, Enterococcus faecium ürediği görülmüş olup prokalsitonin düzeyi 2,26ng/ml bulunmuştur. Olgunun ölüm sebebi yaygın sistemik enfeksiyon olarak düşünülmüştür.

Anahtar Kelimeler

• Toksik Epidermal Nekrolizis
• Escherichia coli
• sepsis
• otopsi

TOXIC EPIDERMAL NECROLYSIS LIKE BULLOUS SKIN REACTION: AN AUTOPSY CASE

Öz

Toxic epidermal necrolysis TEN is characterized by detachment of epidermis from underlying dermis. Vesicular and bullous lesions arise on macules. These lesions rupture easily and eroding lesions remain. Pathogenesis of TEN is not fully clear yet but in general some medications, hepatitis, septicemias of Mycoplasma, Varicella, Herpes, Escherichia coli infections are suspected. Antibiotics, anticonvulsant drugs or nonsteroidal anti-inflammatory drugs are most commonly involved drugs. In some publications mortality rate reported as between 20 and 60 per cent. Most common cause of death is sepsis and subsequent pulmonary involvement in TEN. Enterococcus spp, Acinetobacter spp, Staphylococcus aureus, Pseudomanas aurigonasa are most common causes of sepsis in those cases. A forty year-old man, transported to the hospital in unconscious state without any medical or judicial antecedent and followed in intensive care unit because of cardiac arrest. After one week follow-up patient died and referred to the Council of Forensic Medicine There were widespread except chest and back epidermal bullous lesions and concomitant ruptured bullae with hyperemic and purplecolored underlying tissue. During autopsy there were yellow effusions in chest and abdominal cavity. Lower segments of trachea and esophagus and greater omentum had infective appearance. On the histopathological examination of lungs lobular pneumonia and bronchitis were detected. Severe active enteritis and colitis were detected on small intestine and bowel specimens. On the toxicological screening of blood, 78.4ng/ml Pentobarbital, 169ng/ml Thiopental; Pheniramine, Ranitidine and Metoclopromide were detected. On postmortem microbiological study of blood and cerebrospinal fluid E. coli, Enterococcus faecium; at the lung tissue E. coli, Candida albicans; on the splenic tissue E. coli were colonized. On the microbiological study of abdominal effusion E. coli and Proteus spp were colonized and on bullae fluid E. coli and Enterococcus faecium were colonized. Serum Procalcitonin level was 2.26ng/ml. Cause of death determined as widespread systemic infection.

Anahtar Kelimeler

• Toxic Epidermal Necrolysis
• Escherichia coli
• sepsis
• autopsy

Kaynakça

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- vens–Johnson 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 Allergy Clin Immunol 2013;13 4 :330-6.

9. 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 2 :241-53.
10. Le Cleach L, Delaire S, Boumsell L, Bagot M, Bourgault-Villada I, Bensussan A, Roujeau JC. Blister fluid T lymphocytes during toxic epidermal necrolysis are functional cytotoxic cells which express human natural killer NK inhibitory receptors. Clin Exp Immunol 2000;119 1 :225-30.
11. Nassif A, Bensussan A, Dorothée G, Mami-Chouaib F, Bachot N, Bagot M, Boumsell L, Roujeau JC. Drug specific cytotoxic T-cells in the skin lesions of a patient with toxic epidermal necrolysis. J Invest Dermatol 2002;118 4 :728-33.
12. Cohen S, Billig A, Ad-El D. Ceftriaxoneinduced toxic epidermal necrolysis mimicking 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, BastujiGarin S, Correia O, Locati F, Mockenhaupt M, Paoletti C, Shapiro S, Shear N, Schöpf E, Kaufman DW. Medication use and the risk of Stevens–Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;333 24 :1600-8.
14. Plaut ME, Mirani M. Toxic epidermal 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 revisited. Plast Reconstr Surg 2002;110 3 :768-73.
16. Sekula P, Dunant A, Mockenhaupt M, Naldi L, Bouwes Bavinck JN, Halevy S, Kardaun S, Sidoroff A, Liss Y, Schumacher M, Roujeau JC. Comprehensive survival analysis of a cohort of patients with Stevens–Johnson 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 patient 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 diagnosis of severe cutaneous drug eruptions. Am J Clin Dermatol 2003;4 8 :561-72.