B A O C A O T R I J S N G HOP B $ N H V I E M D A IViyN MU
D\Jd\ LdJP SC/NG (SUB-CORNEAL PUSTULAR DERMATOSIS HAY CON GQI LA B^NH SNEDEX)N-WILKINSON
Dllu TR| TAI BfNH VI^N PHONG DA UEU TRUNG UONG QUY H 6 A
A REPORT OF THE SNEDDON WILKINSON CASE HAVE TREATED IN Q U Y H O A NATIONAL LEPROSY DERMATOLOGY HOSPITAL
Nguyen Thanh Tan*, Nguyen Thi Thoi Loan*
I . G l d l T H l | U
La benh hiem gap, m i n tfnh, t i i p h i t , phat ban kem m u n mD. M d benh hpc d i y d i e te b i o neutrophil. Benh dUOe p h i t hien dau tien nam 1956 bdi Sneddon Wilkinson. D e n n i m 1966, co 130 trudng h o p dUOc bao c i o . Gan d i y ngU6i ta xep benh n i y vao benh IgA p e m p h i g u s n h c f v i o mien djch huynh q u a n g true tiep (tang d g n g IgA t h u o n g bi) [1 ]. Chung t o i xin b i o cao t r u d n g hop benh n i y dieu trj tai Benh vien Phong Da lieu Trung UOng Quy Hoa t h i n g 7 nam 2012.
I I . M Q T A T R l / d N G HOP B l N H
Benh n h i n Nam 29 t u o i , d i n toe: Kinh. Dja chi: Phong Thanh, Tuy PhUdc, Binh D j n h . N h i p vien: 5/7/2012 vi sot cao kem nhieu m u n mCi tren da.
Benh p h i t dau t i e n t h i n g 4 nam 2012, ban dau la c i c d i m da d o , t r o t da, tiet dich, sau do dong v i y tiet, p h i n bo rai rac t o i n than, benh nhan den p h o n g k h i m da lieu t u nhan dMc chan d o i n chdm vi trimg v i duac dieu trj bang
* Benh vien Phong Da liiu Trung uong Quy Hoa.
c i c thuoc k h i n g sinh, corticoid, thuoc boi, t h u o n g t o n da lanh de lai cac dam da nhiem sac.
T h i n g 7 n i m 2012, benh tai phat lai va benh nhan den Benh vien Phong Da iieu Trung uong Quy Hoa. Benh nhan den vien trong tinh trang met, sot cao 40°C, mach 120 chu ki/phut, huyet i p 120/70 m m H g . Benh n h i n nang 60kg.
Thuong t o n da l i m u n mCi, bong mCi, bpng nUde, don doc v i thanh dam tren nen da do, m o t so t h u o n g t o n xuat hien trUdc thi dap vd, c h i y nUdc, d o n g vay tiet ban tao thanh d i m kho nut, ben dudi am udt chu'a dich mu, mot so CO khuynh hUdng lanh d t r u n g t i m v i thuong ton mdi xuat hien t h e m d ngoai vi.
Thuong t o n sap xep thanh hinh dang dong tien, t h i n h d i m , phan bo ehCi yeu d mat duoi hai tay, hai n i c h , bung, rai rae hai c h i n . Tai vj trf mat, lung, long ban tay, b i n c h i n khong thay t h u o n g t o n . (Hinh 1,2,3,4) Toan t h i n benh n h i n met do sot nhung t o i n trang khong g i y sut.
Trieu chiJng co n i n g khong ngijfa, dau r i t it d t h u o n g t o n .
Lay benh pham xet nghiem trUdc khi diing thude (5/7/2012): a M co BC: 25.400, N:69%, L:28%, HC:5,2 trieu, Hb;121000, TC:200.000.
S6 08 (Thang 11/2012) D A L I ^ U H O C I 5 5
DiSn D^n
HbsAg, anti HCV, HIV cho k^t q u i i m tinh. SCOT:
40, SGPT:35 Ul/L, ure miu: 3,9mmoI/L, creatlnin miu: 135Mmot/L, glucose miu: 5,7mmot/L.
Oi^n giii 66: binh thUdng. T6ng phin tfch nude tl^u: binh thudng. Ol&n t i m 66, X-quang tim ph6l, SiSu i m t6ng quit 6 bgng: binh thudng. T^ bio Tzanck: khdng tim thiy t^
bio gai t^eh hinh v i t^ bio da nhin kh6ng 16.
Nhudm Gi^m sa mun mO thiy t^ bio Neutrophil chi^m 98%, Eosinophil chi^m 2%. (hinh 11).
Nudi ciy mil: khdng thiy vi khuin giy b&nh mpc. Ket q u i md b^nh hpc md t i : ThUOng bl c6 bpng mun nude n i m ngay dUdi Idp sC/ng, trong bong mgn nUde cd nhl^u bach ciu da nhin trung tfnh. Trung b\: xim nhip viem bach ciu da nhin trung tfnh v i bach cau don nhin quanh cae huyet quin v i trung bl ndng. (hinh 9,10)
III.CHANDOAN
1. Chan doan: Viem da mun mu dUdi Idp sUng (Subcorneal pustular dermatosis hay Sneddon Wilkinson).
2. Chan doan phdn bift: Benh choc, viSm da dang herpes, hdi chUng Sweet, chim vi trung phin Ung thU phit, phit ban mun mO cap tfnh (acute generalized exanthematous pustulosis), viy nen the mCi.
IV. D i i u TR!
1. Dieu trj cu th^
Djch truyen boi phi^ nude dien giit.
• Glucose 5% 500ml, natrichtorua 0,9%
500mt, ringertaetate lOOOmt/ngiy
• Kaliehloride 10% 1 g x 2 dng / ngiy Corticosteroid:
• Sotumedron 40mg x 3 Ip/ngiy (2mg/kg/
ngay) X10 ngay
Sotumedron 40mg x 11/2 lo/ngiy (Img/
kg/ngay) x 5 ngiy
• Sotumedron 40mg x 1 /2 Ip/ngiy x 5 ngiy
• Medron 16 mg x 10 ngiy (dOn ngoai trii) Thu5c h6 tr(? tri^u chiJng khic: ha sot, thudc b6.
Chim sdc da tai chd: chSm mun bpng nudc bing milian, d i p gac Jarlsh d thuong t6n ti^t djch, sau khi thuong t6n khd bdi kem triamcinolone.
2. Tl^n trl^n v i 6ip uTng di£u trj Cit sdt sau 4 gid di^u trj.
Khdng xuit hi^n th^m thuong t6n mdi sau 2 ngiy dl^u trj.
Thuong tdn khd, het tiet djch, ddng viy tiet khd sau 10 ngiy diiu tri.
Sach thuong ton hoin t o i n sau 20 ngiy, benh nhin xuit vien, dieu trj ngoai trO, benh nhin t i l khim lai sau khi het thudc dieu trj ngoai tru, benh khdng t i i phit.
V. BAN LUAN 1. VechSndoan
Viem da mun mCi dudi Idp si/ng cd bieu hien tim sing l i mun mCi, mun nUdc nhd xuat hien tren nin da dd hay binh thudng, nhanh chdng lan tda trong v i l gid. Mun mCi, mun nUdc eo khuynh hudng ti^n kit, t i p trung thinh hinh ddng tien, hinh lUdi liem hay theo dudng day t h i n kinh (serpiginous).
Sau vai ngay mun mill vd, khd, dong viy tiet gidng nhU chde. D i m thuong ton cd khuynh hudng khd xep tinh giCfa trong khi bd ngoai vi thuong ton dd v i them mpc mun mD mdi. Khi linh thuong ton khdng thanh seo ma de lai dit nhiem sic td. Hay khi khim phit hien cic dim nhiim sSc cCia thuong ton trUdc diy. Khdng dieu trj vai ngiy v i l tuan tai xuat hien thUong ton mdi.
IVIun mO tren nen ban dd cd xu hUdng xuat hiln dot xufng hai ben chCi y l u d nach, ben, bung, dudi vu, nep gap cua tay, chin. TrUdng
5 6
I DA LII^U HOC S6 08 (Thing 11/2012)Dien Dan
hop hiem xuat hien tren mat, tdng b i n tay, b i n c h i n . Da dau va n i e m mac k h d n g bi t h u o n g t o n . Trieu chUng ngCfa v i b d n g r i t ft. M d benh hpc:
mun mCi d u d i Idp siing va x a m n h i p v i e m day d i e BC da n h i n t r u n g t f n h , rat ft BC i i toan. Cd it te b i o ty gai t i m t h a y 6 t h u o n g t o n thuT p h i t . ThUdng bl cd x d p b i o . T r u n g bl x i m n h i p v i l m quanh mach m i u chu yeu la BC da nhan t r u n g tfnh, hiem khi t h i y BC d o n nhan v i i i toan.
Mien djch huynh q u a n g trUe tiep: t i n g d o n g IgA thuong bl (intraepidermal) [1][2][3]
d benh n h i n n i y , vdi t i l n sU, benh sU, bleu hien tam s i n g va can tam sang c h u n g tdi c h i n d o i n loai trii c i c benh choc, v i e m da dang herpes, hoi chUng Sweet, cham vi trUng p h i n Lfng thU p h i t , p h i t ban m u n m i l t o a n t h i n cap tfnh (acute generalized exanthematous pustulosis), v i y nen t h i mCi.
Benh choc, khdi phat la b o n g nUde, hda mu nhanh, bpng nUdc d i p v d c h i y dich tan den dau thi t h u o n g t o n m d i xuat hien den d o , sau do dong v i y tiet v i n g a m , vj trf d viling dau nhieu Hon viJng khac cCia co t h i , t h u d n g gap d t r i em cd ve sinh kem, nuoi cay m u cd t u cau hay lien cau. Viem da d a n g herpes t h u o n g t d n l i san d d , mun nudc t h a n h n h d m , d i p v d v i xen tan vet cao gai, ddi khi cd b g n g nUdc n h u n g khdng ed thuong t o n mCi kem t h e o ngiJa.
Md benh hgc m u n nUde dUdi thUdng bl, vi i p xe d nhii bl chCi y e u ta bach c i u da n h i n t r u n g tfnh va bach c i u Ua acid d nhu bl. Hdi ehUng Sweet t h u d n g g a p 6 nCf h d n n a m , thUdng t o n la s i n do, m i n g d d , t a p t r u n g t h a n h n h o m ed hinh ddng t i e n hay hinh tudi tiem, b d r d , khdng deu, phiJ ne, v i l m dau, t h u o n g t o n cd tfnh chat t i n h giCra va tan ra ngoai v i , b d noi cao; cd t h e CO bpng nUdc, m u n mO, b e n h t h u d n g bieu hien viJng mat, cd trUdng h p p t u p h i t nhUng cd trudng h g p benh lap sau b e n h ty khdi u. C h i m vi triing p h i n i ^ g t h U phat, t h u d n g la tU 6 nhiem khuan dau t i l n nhU v i l m tai, nhiem t r i i n g da
d o chan t h u o n g , con t r i i n g can, t h u o n g tdn ndi m u n nudc v i ngUa ten sau d d p h i n u'ng ngUa t o i n t h i n kem theo c h i m hda va nhiem trting da d o c i o g i i .
Benh p h i t ban m u n mu toan than cap tfnh (acute generalized exanthematous pustulosis - AGEP), la benh p h i t ban do thuoc dang mun mCi, do p h i n Ung giOa co the vdi mot sd loai thudc n h u khang sinh n h d m betatactamin, earbamazepine, atlopurlnot, sulfonamides... khi nhiem m o t sd virus n h u EBV, CMV,... V i y nen the m u , tren nen cCia benh v i y nen, do dieu trj khdng dCing c i c h hay do nguyen n h i n nao dd tam xuat hien m u n m u t h i n h d i m tren nen da d d , mun mCi ndng rop nhung khdng cd bpng m u n nUdc, ed the t o i n than hay khu t r u . Md benh hoe ed m u n mu dang xop cua Koji. [1][2][3].
Vdi bieu hien lam sang va m d benh hoc ciing vdi ket q u i dieu trj benh, vdi phuong phap chan d o i n loai trif, mac d i i chung toi chua cd mien dieh huynh quang trUe tiep de xae djnh nhUng cho phep chung tdi nght nhieu den chan doan benh nay la viem da m u n mu dUdi Idp sifng hay l i benh Sneddon Wilkinson. Benh khong de chan doan ngay tii d i u khi mot so bac sT chuyen khoa trude d d da chan doan vdi cae benh khac nhau: eham vi t r i i n g , viem da cd mCi, viem da do nhiem triing...
2. V l d i e u t r i
Thude dieu tri dau t i l n cho benh v i l m da m u n mil dUdi Idp sUng l i : sutfone 50-150mg/
n g i y , c o r t i c o i d . T h u d c t h a y t h e k h i c la retinoids, cotchicin, cyclosporin A [1]. 6 benh n h i n nay chung tdi sii d u n g corticoid v i c i c thudc dieu tri trieu chUng khac. Benh d i p uYig vdi d i i u trj, eat sot sau 4 g i d dieu t r i , khdng xuat hien t h e m t h u o n g t o n m d i sau 2 ngay dieu t r i . ThUdng t d n khd, het tiet dich, d d n g v i y tiet khd sau 10 n g i y dieu trj. Sach t h u o n g t o n hoan toan sau 20 n g i y {hinh 5,6,7,8), benh nhan xuat vien, dieu
S6 08 (Thang 11/2012) D A L I ^ U H O C I
57
DiSn ein
- » - • - •
tri ngoai tru, b§nh nhSn teii kh^m l^i sau khi li^t thu6c di^u tr! ngo^i trii, Wnh kliftng ti\ pliSt.
Tidn li/ang b&nh kli5 quan nhung chiing tdi vin ti^p tuc theo doi vS thirdng xuy^n li&n h$
v6i b$nh nhan d*' xem b^nh c6 t j i phSt khdng.
Trong quei trinh di4u trj, b$nh d6p ijrng t6t.
TAILICUTHAMKHAO
and
khdng c6 diSn biSn bSt thudng gi, chung tdi chi h<f[ chSn m^t ISn d^ thdng nhSt chSn doin vS ph^c dd dl^u tri:
Hy vgng trudng hap b^nh ndy cung gdp phdn chiasm vdiquf ddng nghi$p v^kinh nghiSmchdn dodn vd diiu trj b$nh da II gdp.
3. Rook's testbook of dermatology. Sneddon Wilkinson. Seventh edi.2010.
1. Fitzpathcif's Clinical Dermatology medicine. Sixth edi, 2009.
2. John Hunter. Clinical dermatology. Sneddon Wilkinson. Thirdedi.20W
HlNH ANH BENHNHAN
Benh nhin Nam 29 tu6i, d i n toe kinh. Dja chi: Phong Th?nh, Tuy Phudc, Binh Djnh. Nhip vien:
5/7/2012 vl sdt cao kem nhieu mun mu trin da.
Hinh 1. Bong nude, bong mO dan ddc vd thdnh ddm phdn bd ddy ddc 6
cdnh cdng tay.
Hinh 2. Mun mii, bpng mii, bgng nude, dan ddc vd thdnh ddm trin nin da dd. s6 moc trudc ddp vd, chdy nUdc, ddng vdy tiit bdn
tgo thdnh ddm khd nUt. bin dudi dm udt dich mij, vi tridhdng bung trdi.
Hinh 3. Bgng nUdc khong 16, nong, nhdn nheo, lan tda, de chat vd, xung quanh
nhieu bgng nude nhd vi tri d chdn.
Hinh 4. Bgng nudc, bgng mO thdnh ddm ddy dde d v^ng ndch.
5 8 I DA Ll£U H p c s6 oe (ThSng 11/2012)
Hinh Snh benh nhan sau dieu tri 20 ngay, cdc thUcmg ton cliup tai vi tri trade dieu trj de so sanh.
Hinh 5. r/ii.(v).ii,...uui,iMi s.ji
dieu In de lai :,ev nun \iii lo iliiHinh 6. :hti"!i<i (i>n ilhinh seoxiit!
vung hong bung.
Htnh 7. Thuang ton da sau dieu tri vung bung delglseo dd thdm.
Hinh 8. Thuang ton sau dieu tn vung ndch delgi ddm thdm da.
J r ^ > ^ * *f'^''
m - -fir
Hinh 9va10. Thuang bi cd bong mun nudc ndm ngay dudi Idp Hinh 11. Nhudm Giim sa dich simg, trong bong mun nudc cd nhieu bach cdu da nhdn trung tinh mu: tebdo Neutrophil chiem Uu Trung bi; xdmnhdp viem bach cdu da nhdn trung tinh vd bach cau the, tebdo Eosinophil rat
dan nhdn quanh cdc huyit qudn vd trung bi ndng. it ithdng ddng ke.
S6 08 (Thang 11/2012) DA LIEU HOC 5 9
