En Coup De Sabre-Guttat Morfea Birlikteliği - Yeni Tıp Dergisi
En Coup De Sabre-Guttat Morfea Birlikteliği - Yeni Tıp Dergisi
En Coup De Sabre-Guttat Morfea Birlikteliği - Yeni Tıp Dergisi
Create successful ePaper yourself
Turn your PDF publications into a flip-book with our unique Google optimized e-Paper software.
gisi 2013;30(Suppl 1):33-35<br />
Olgu sunumu<br />
Nadir Bir Lokalize Skleroderma Olgusu: <strong>En</strong> <strong>Coup</strong><br />
<strong>De</strong> <strong>Sabre</strong>-<br />
1 ,Aynur SOLAK<br />
2 3<br />
1 <br />
2 <br />
3 <br />
ÖZET<br />
Bu makalede lineer skleroderma “en coup de sabre” ve<br />
-<br />
<br />
ldiril-<br />
<br />
literatürü gözden geçirdik.<br />
Anahtar Kelimeler: Skleroderma; lokalize; hipokampus;<br />
atrofi<br />
<br />
<br />
-<br />
<br />
-<br />
deriye lokalize (lokalize skleroderma) de<br />
olabilir. Lineer skleroderma “en coup de sabre”<br />
(LSCS)’de lezyon tipik olarak paramedian pozis-<br />
<br />
<br />
-<br />
-<br />
<br />
<br />
<br />
inflamatuar, 2-<br />
plaklarla kendini gösterir 1,2 .<br />
LSCS <br />
Manyetik Rezonans Görüntülemede (MRG) hipo-<br />
<br />
sunduk. Literatür bilgilerimize göre bu üç bulgu<br />
<br />
OLGU<br />
Otuzdört <br />
<br />
<br />
benzeri <br />
ve aile hikayesinde herhangi bir özellik yoktu.<br />
<br />
D<br />
<br />
e-mail: belkisuyar@gmail.com<br />
: 20.12.2011<br />
: 19.01.2012<br />
ABSTRACT<br />
An unusal case of localised scleroderma: coexistence<br />
of en coup de sabre and guttate morphea<br />
In this case report, we present a 34 year-old woman<br />
who had linear scleroderma ‘’en coup de sabre’ and<br />
guttate morphea with hippocampal atrophy in the cranial<br />
magnetic resonance imaging by reviewing the literature,<br />
since all the three symptoms were not reported before in<br />
a single case.<br />
Key Words: Scleroderma; localized; hippocampus; atrophy<br />
<br />
, atrofik,yara<br />
(Resim 1).<br />
Resim 1. , hipopigmente, atrofik<br />
lezyonlar<br />
Resim 2. Boyun bölgesinde lineer ve oval 3-8 mm boyutunda<br />
atrofik lezyonlar<br />
<br />
çökme eer ve oval<br />
3- -6 adet benzer lezyonlar<br />
<br />
ASO, CRP, RF, idrar tahlili, eritrosit sedimantasyon<br />
TSH , Anti<br />
33
B. Uyar ve ark.<br />
dsDNA, TPHA, RPR, Borrelia burgdorferi IgM ve<br />
IgG negatifti. Hemoglobin 7,8 g/dL, ferritin 3<br />
ng/ml idi.<br />
Hastaya, morfea, anotoderma, atrofoderma,<br />
<br />
kapsül (ferrosanal duodenal kapsül) 2x1, kalsipotriol<br />
pomat (Psorcutan pomat) 2x1 <br />
<br />
<br />
Palpasyonda<br />
<br />
<br />
<br />
lezyonlara yönelik dijital probla (Siemens Acuson<br />
Antares, VF 13-5SP, intraoperative transducer)<br />
Ai lezyonlarda,<br />
3 <br />
-<br />
<br />
<br />
lezyonda sonografik olarak periosteal çöküntü<br />
sapt <br />
<br />
<br />
<br />
<br />
hipokampal atrof <br />
<br />
<br />
<br />
Resim 3. (A) Ultrasonografik görüntülemede hipoekoik<br />
<br />
<br />
tedir<br />
Resim 4. <br />
hafif<br />
dilatasyon izlenmektedir<br />
<br />
-<br />
<br />
ve eklerinde kaybolma, kollajen bantlarda artma<br />
di (Resim 5).<br />
Yu -<br />
<br />
du. Hastaya 4 hafta süreyle sefuroksim<br />
sodyum 500 mg tbl 2x1, Kalsipotriol pomat 2x1,<br />
vitamin E 300 mg 2x1, sentetik antimalarial olan<br />
hidroksikolorokin sülfat 200 <br />
Resim 5. <br />
-<br />
Eozin X40<br />
-<br />
lik<br />
<br />
<br />
<br />
organ tutulumuna yol açmayan, yüzeysel formu-<br />
- <br />
<br />
götser<br />
<br />
<br />
<br />
rarak<br />
lokalize olmaya meyillidir. Jeneralize guttat<br />
morfea cuttur<br />
2 .<br />
<br />
-8 mm lik, deri rengine<br />
<br />
<br />
teren lineer skleroderma-<br />
<br />
<br />
veya fronto- <br />
<br />
görülen klinik bulgudur. Lezyonlar burun, yanak,<br />
3,4 <br />
-<br />
LSCS<br />
<br />
LSCS ile birlikte sistemik bulgu <br />
nörolojik komplikasyonlar görülür. Nörolojik bulgu<br />
34
B. Uyar ve ark.<br />
-<br />
5 . <br />
<br />
-<br />
<br />
<br />
6 . Marzano ve ark.<br />
bildirdikleri 239 lokalize skleroderma hastas<br />
17 hastada <br />
<br />
oral ve nörolojik tutulum mevcuttur 7 .<br />
<strong>De</strong>ri bulgular <br />
<br />
kaç ay sonra görülür. Fakat bir<br />
<br />
bulgularla seyreden vakalar 8,9 .<br />
-<br />
-<br />
10 . Helene E. ve ark. hipokampal atrofi ve<br />
<br />
6 Sistemik<br />
<br />
9 .<br />
Nörolojik bulgusu olmayan, epilepsi nöbetleri<br />
tariflemeyen hastalarda bile anormal MRG ve<br />
11,12 .<br />
-<br />
<br />
luklarla<br />
kendini gösterir. Öyküsünde tipik epilepsi<br />
anemnezi vermeyen olgumuzun nörolojik muaye-<br />
<br />
<br />
8,13 .<br />
kimal kalsi-<br />
<br />
1. Krieg T. Scleroderma. in: Braun-Falco O, Burgdorf WHC, Plewig G,<br />
Wolff HH, Landthaler M, editors. 3th ed. Braun Falco’s <strong>De</strong>rmatology. Italya:<br />
Springer Medizin Verlag Heidelberg; 2009. p. 701-15.<br />
2. Blaya B, Gardeazabal J, Martínez de LagránZ, Díaz-Pérez JL.<br />
Patient With Generalized <strong>Guttat</strong>e Morphea and Lichen Sclerosus et<br />
Atrophicus. Actas <strong>De</strong>rmosifiliogr 2008;99:808-11.<br />
3. Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de<br />
sabre) following Blaschko's lines. J Am Acad <strong>De</strong>rmatol 1998;38:366–8.<br />
4. Itin PH, Schiller P. Double-lined frontoparietal scleroderma en coup<br />
de sabre. <strong>De</strong>rmatology 1999;199:185–6.<br />
5. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M,<br />
Espinoza LR. Linear scleroderma en coup de sabre and brain calcification: is<br />
there a pathologic relationship J Rheumatol 2003;30:193–5.<br />
6. Verhelst HE, Beele H, Vanneuville RB, VanCoster RN. Hippocampal<br />
atrophy and developmental regressionas first sign of linear scleroderma ‘‘en<br />
coup de sabre’’. European Journal of Paediatric Neurology 2008;12:508–11.<br />
7. Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, et al.<br />
Localized scleroderma in adults and children: clinical and laboratory<br />
investigations of 239 cases. Eur J <strong>De</strong>rmatol 2003;13:171–6.<br />
8. Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma “en coup<br />
de sabre”: pathological evidence of intracerebral inflammation. J Neurol<br />
Neurosurg Psychiatry 2001;70:382–5.<br />
9. Menni S, Marzano AV, Passoni EP. Neurologic abnormalities in two<br />
patients with facial hemiatrophy and sclerosis coexisting with morphea.<br />
Pediatr <strong>De</strong>rmatol 1997;14:113–6.<br />
10. Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet<br />
BA, et al. Linear Scleroderma en coup de sabre With Associated Neurologic<br />
Abnormalities. Pediatrics 2006;117: e132<br />
11. Liu P, Uziel Y, Chuang S, Silverman E, Krafchik B, Laxer R.<br />
Localized scleroderma: imaging features. Pediatr Radiol 1994;24:207–9.<br />
REFERANSLAR<br />
<br />
radyolojik bulgular da izlenebilmektedir 8,11,12,14,15 .<br />
<br />
nik olarak<br />
düzelebilir. <br />
<br />
<br />
-sklerotik<br />
<br />
lezyonlardaki kemik defektini, yeni lezyonlardaki<br />
periostal kesilme ve inflamatuar periostiti göster-<br />
<br />
olgumuzun nörolojik muayenesinde patolojik bul-<br />
<br />
Sistemik, topikal ve lezyon içi kortizon tedavisi<br />
syonu azaltabilmekte-<br />
-<br />
<br />
<br />
aminobenzoat potasyum, penisilin, retinoidler,<br />
difenilhidantoin, interferon- unsupressan<br />
ilaçlar gibi sistemik ajanlar ve ultraviole-A tedavi-<br />
<br />
hydroxychloroquine, D-penisilamin, methotrexate,<br />
cyclosporine, and cyclophosphamide <br />
ekil<br />
-rekonstrüktif<br />
13,16-20 .<br />
<br />
<br />
uzun süreli ve dikkatli takip etmek gerekir.<br />
Etyolojisi ve patogenezi tam olarak bilinmeyen bu<br />
<br />
<br />
<br />
<br />
12. Appenzeller S, Montenegro MA, <strong>De</strong>rtkigil SS, Sampaio-Barros PD,<br />
Marques-Neto JF, Samara AM, et al. Neuroimaging findings in scleroderma<br />
en coup de sabre. Neurology 2004;62:1585–9.<br />
13. Obermoser G, Pfaulser BE, Linder DM, Sepp NT. Scleroderma en<br />
coup de sabre with central nervous system and ophthalmologic<br />
involvement: treatment of ocular symptoms with interferon gamma. J Am<br />
Acad <strong>De</strong>rmatol 2003;49:543–6.<br />
14. Unterberger I, Trinka E, <strong>En</strong>gelhardt K, Muigg A, Eller P, Wagner M,<br />
et al. Linear scleroderma “en coup de sabre” coexisting with plaquemorphea:<br />
neuroradiological manifestation and response to steroids. J<br />
Neurol Neurosurg Psychiatry 2003;74:661–4.<br />
15. Heron E, Hernigou A, Chatellier G, Fornes P, Emmerich J,<br />
Fiessinger JN, et al. Intracerebral calcification in systemic sclerosis. Stroke<br />
1999;30:2183–5.<br />
16. Eubanks LE, McBurney EI, Galen W, Reed R. Linear scleroderma in<br />
children. Int J <strong>De</strong>rmatol 1996;35:330–6.<br />
17. Cunningham BB, Landells ID, Langman C, Saliler DE, Paller AS.<br />
Topical calcipotriene for morphea/linear scleroderma. J Am Acad <strong>De</strong>rmatol<br />
1998;39:211–5.<br />
18. Hawk A, <strong>En</strong>glish JC 3rd. Localized and systemic scleroderma.<br />
Semin Cutan Med Surg 2001;20:27–37.<br />
19. Elst EF, Van-Suijlekom-Smit LW, Oranje AP. Treatment of linear<br />
scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven<br />
children. Pediatr <strong>De</strong>rmatol 1999;16:53–8.<br />
20. Uziel Y, Feldman BM, Krafchik BR, Yeung RS, Laxer RM.<br />
Methotrexate and corticosteroid therapy for pediatric localized scleroderma.<br />
J Pediatr 2000;13:91–5.<br />
35