ºÝºÝߣ

ºÝºÝߣShare a Scribd company logo

Durhing ¨C bro cq

?Download as PPTX, PDF?
?
T
Tr?n B¨¢ Tr¨ª

da lieu

1 of 20
DURHING - BROCQ ? B?nh da b?ng n??c t? mi?n c? li?n quan ??n b?nh t/c m? v?i t? ch?ng Brocq: +. T?n th??ng ?a d?ng: h?ng ban, b?ng n??c + D?u hi?u ch? quan r? + Ti?n tri?n t?ng ??t + To?n tr?ng t??ng ??i t?t
? D?ch t? h?c: + B?nh g?p ? m?i l?a tu?i, th¨ºng g?p t? 2 - 90 tu?i, tu?i kh?i ph?t thu¨ºng l? 40 + ? VN s¨¨ l??ng b?nh nh?n g?p ? d??i 5 tu?i chi?m 40.7%
? L?m s¨¤ng: + Th? tr?ng b¨¬nh th??ng, s?t nh? or m?t m?i Ng?a l¨¤ ti?n tri?u + T?n th??ng da: h?ng ban, s?n ph¨´ sau th¨¤nh b?ng n??c, b?ng n??c xu?t hi?n tr¨ºn n?n da ??, ??ng l? t? ho?c ch?m l?i nh? trong b?nh herpes n¨ºn b?nh c¨°n ???c g?i l¨¤ vi¨ºm da d?ng herpes
? ??c ?i?m b?ng n??c: + B?ng h?t ng?, c?ng, tr¨°n, b¨®ng, trong ch?a d?ch m¨¤u v¨¤ng chanh xung quanh c¨® qu?ng ?? (hi?m khi c¨® b?ng n??c xu?t huy?t). Sau 5- 7 ng¨¤y b?ng n??c chuy?n sang b?ng n??c: m¨¤u ??c do b?i nhi?m + Sau v¨¤i ng¨¤y b?ng n??c v? ra ?? l?i v?t tr?t, ?¨®ng v?y ti?t (?en), v?y m? (v¨¤ng ??c)
+ V? tr¨ª th??ng t?n: khu?u tay,??u g?i,m?ng, l?ng, b?ng, ?¨´i, ¨ªt khi g?p ? k? n¨¢ch v¨¤ v¨´ng x??ng c¨´ng c?t, c¨® t¨ªnh ch?t ??i x?ng + Tr¨ºn c¨´ng m?t v? tr¨ª c¨® th? g?p nhi?u tt c¨´ng m?t luc: h?ng ban,b?ng n??c, s?n ph¨´¡­ + TTNM: ¨ªt g?p (4,6%) + DH NISKOSKY : ?M T?NH + ?t khi th?y n?i h?ch
? Ti?n tri?n: + B?nh ti?n tri?n m?n t¨ªnh: l¨²c t?ng, l¨²c gi?m + C¨® th? t¨¢i ph¨¢t, nh?ng h?n ch? ???c b?ng DDS ho?c ch? ?? ?n ko c¨® gluten + B?nh ¨ªt ?nh h??ng ??n sinh ho?t lao ??ng c?a b?nh nh?n( tr? tr??ng h?p ng??i cao tu?i, suy ki?t¡­)
? Nguy¨ºn nh?nb?nh sinh: + Y?u t? mi?n d?ch: + Y?u t? di truy?n + Li¨ºn quan ??n b?nh t? mi?n + Vai tr¨° gluten: ?n ch? ?? c¨® gluten l¨¤m v??ng b?nh l¨ºn + Li¨ºn quan ??n b?nh ti¨ºu ch?y m? + Li¨ºn quan ??n b?nh kh¨¢c v¨¤ u ¨¢c t¨ªnh
Y?u t? di truy?n HLA-A1, HLA-B8, HLA-DR3, v¨¤ HLA-DQ2. HLA-B8, li¨ºn quan t?i 58-87% HLA-DR3, li¨ºn quan t?i 90-95 HLA-DQ2, li¨ºn quan t?i 95-100% + M?t s? t¨¢c gi? ghi nh?n tr??ng h?p sinh ??i c¨´ng tr?ng, trong ?¨® m?t m?c DH, ng??i kia m?c b?nh vi¨ºm ru?t. V?y n¨ºn, y?u t? m?i tr??ng c?ng ?¨®ng vai tr¨° quan tr?ng trong qu¨¢ tr¨¬nh h¨¬nh th¨¤nh b?nh.
? C?n l?m s¨¤ng: + XNM: bc ¨¢i toan t?ng 10% + Test KI: cho ng??i b?nh u?ng KI 1g li?u duy nh?t test ¨¢p t?i ch? m? KI 50% v?i vaselin l¨ºn v¨´ng da b¨¬nh th??ng. ??c test sau 24 ? 48 gi? v¨´ng da th? ng?a, n?i ban ?? ho?c xh b?ng n??c. + MBH: b?ng n??c d??i th??ng b¨¬, ko v¨® hi?n t??ng teo gai, ??nh nh¨² b¨¬ th?m nhi¨ºm BC?N TT
? C?n l?m s¨¤ng: + MDHQ: ti¨ºu chu?n v¨¤ng MDHQTT: l?ng ??ng IGA d?ng H?T ? nh¨² b¨¬ (ranh gi?i gi?a trung b¨¬ v¨¤ h? b¨¬). Ngo¨¤i ra c¨°n th?y C3 v¨¤ Fibrinogen. MDHQGT: 70% IGA l?u h¨¤nh trong m¨¢u
? Ch?n ?o¨¢n x¨¢c ??nh: + Th??ng t?n ?a d?ng: b?ng n??c, m?n n??c, s?n, s?n ph¨´, d¨¢t ?? + B?ng n??c c¨® qu?ng ?? xung quanh, t?p trung th¨¤nh t?ng c?m tr¨ºn n?n d¨¢t ?? + C¨® ti?n tri?u ng¨²a, t¨¢i ph¨¢t, tt b¨¬nh th??ng + Test KI 50% (+) tr¨ºn 85% b?nh nh?n + MBH: b?ng n??c d??i th??ng b¨¬ + MDHQ: l?ng ??ng Ig A d?ng h?t ? nh¨² b¨¬
? Ch?n ?o¨¢n PB: + H?ng ban ?a d?ng + Ch?c + Pemphigus, + Pemphigoid + B?nh zona, + herpes + B?nh ly th??ng b¨¬ b?ng n??c b?m sinh + Ig A th¨¤nh d?i
?i?u tr?: + T?i ch?: ch? y?u d¨´ng dd s¨¢t khu?n t?i ch?: xanhmethylen, millian. N?u b?ng n??c to th¨¬ ch?c v? ?? l?i m¨¤ng v¨¤ ch?m dd sk.TT kh? d¨´ng cort v¨¤ ks b?i. + Thu?c t¨ªm pha 1/10.000 t?m
+ To¨¤n th?n: - Ch? ?? ?n ko c¨® gluten, h?n ch? ?n iod v¨¤ ?? bi?n - DDS 100-300 mg/ng¨¤y, ?? sau v¨¤i ng¨¤y.D¨´ng duy tr¨¬ ?? h?n ch? t¨¢i ph¨¢t - Sulfapyridin d¨´ng thay th? khi ko ?¨¢p ?ng DDS 1-1,5g/ng¨¤y _ C¨® th? dung cort v?i ks nh¨®m cyclin,
Abstract: We report a case of dermatitis herpetiformis co-localised with segmental vitiligo in a 37-year-old woman with a background history of autoimmune polyglandular syndrome type 2. We propose genetic mosaicism as a possible mechanism. There has only been one previous case report in which dermatitis hepetiformis co-localised in close proximity but not exclusively within vilitigo in a patient with autoimmune thyroiditis. To our knowledge, this is the first case report of dermatitis herpetiformis co- localised exclusively to segmental vitiligo in the presence of autoimmune polyglandular syndrome. KEYWORDS: Addison¡¯s disease; Duhring's disease; autoimmune hypothyroidism; autoimmunity; coeliac disease; dermatitis herpetiformis; diabetes mellitus; vitiligo
Durhing ¨C bro cq
Durhing ¨C bro cq
Durhing ¨C bro cq
Durhing ¨C bro cq
THANK YOUR WACHING!

More Related Content

Durhing ¨C bro cq

  • 1. DURHING - BROCQ ? B?nh da b?ng n??c t? mi?n c? li?n quan ??n b?nh t/c m? v?i t? ch?ng Brocq: +. T?n th??ng ?a d?ng: h?ng ban, b?ng n??c + D?u hi?u ch? quan r? + Ti?n tri?n t?ng ??t + To?n tr?ng t??ng ??i t?t
  • 2. ? D?ch t? h?c: + B?nh g?p ? m?i l?a tu?i, th¨ºng g?p t? 2 - 90 tu?i, tu?i kh?i ph?t thu¨ºng l? 40 + ? VN s¨¨ l??ng b?nh nh?n g?p ? d??i 5 tu?i chi?m 40.7%
  • 3. ? L?m s¨¤ng: + Th? tr?ng b¨¬nh th??ng, s?t nh? or m?t m?i Ng?a l¨¤ ti?n tri?u + T?n th??ng da: h?ng ban, s?n ph¨´ sau th¨¤nh b?ng n??c, b?ng n??c xu?t hi?n tr¨ºn n?n da ??, ??ng l? t? ho?c ch?m l?i nh? trong b?nh herpes n¨ºn b?nh c¨°n ???c g?i l¨¤ vi¨ºm da d?ng herpes
  • 4. ? ??c ?i?m b?ng n??c: + B?ng h?t ng?, c?ng, tr¨°n, b¨®ng, trong ch?a d?ch m¨¤u v¨¤ng chanh xung quanh c¨® qu?ng ?? (hi?m khi c¨® b?ng n??c xu?t huy?t). Sau 5- 7 ng¨¤y b?ng n??c chuy?n sang b?ng n??c: m¨¤u ??c do b?i nhi?m + Sau v¨¤i ng¨¤y b?ng n??c v? ra ?? l?i v?t tr?t, ?¨®ng v?y ti?t (?en), v?y m? (v¨¤ng ??c)
  • 5. + V? tr¨ª th??ng t?n: khu?u tay,??u g?i,m?ng, l?ng, b?ng, ?¨´i, ¨ªt khi g?p ? k? n¨¢ch v¨¤ v¨´ng x??ng c¨´ng c?t, c¨® t¨ªnh ch?t ??i x?ng + Tr¨ºn c¨´ng m?t v? tr¨ª c¨® th? g?p nhi?u tt c¨´ng m?t luc: h?ng ban,b?ng n??c, s?n ph¨´¡­ + TTNM: ¨ªt g?p (4,6%) + DH NISKOSKY : ?M T?NH + ?t khi th?y n?i h?ch
  • 6. ? Ti?n tri?n: + B?nh ti?n tri?n m?n t¨ªnh: l¨²c t?ng, l¨²c gi?m + C¨® th? t¨¢i ph¨¢t, nh?ng h?n ch? ???c b?ng DDS ho?c ch? ?? ?n ko c¨® gluten + B?nh ¨ªt ?nh h??ng ??n sinh ho?t lao ??ng c?a b?nh nh?n( tr? tr??ng h?p ng??i cao tu?i, suy ki?t¡­)
  • 7. ? Nguy¨ºn nh?nb?nh sinh: + Y?u t? mi?n d?ch: + Y?u t? di truy?n + Li¨ºn quan ??n b?nh t? mi?n + Vai tr¨° gluten: ?n ch? ?? c¨® gluten l¨¤m v??ng b?nh l¨ºn + Li¨ºn quan ??n b?nh ti¨ºu ch?y m? + Li¨ºn quan ??n b?nh kh¨¢c v¨¤ u ¨¢c t¨ªnh
  • 8. Y?u t? di truy?n HLA-A1, HLA-B8, HLA-DR3, v¨¤ HLA-DQ2. HLA-B8, li¨ºn quan t?i 58-87% HLA-DR3, li¨ºn quan t?i 90-95 HLA-DQ2, li¨ºn quan t?i 95-100% + M?t s? t¨¢c gi? ghi nh?n tr??ng h?p sinh ??i c¨´ng tr?ng, trong ?¨® m?t m?c DH, ng??i kia m?c b?nh vi¨ºm ru?t. V?y n¨ºn, y?u t? m?i tr??ng c?ng ?¨®ng vai tr¨° quan tr?ng trong qu¨¢ tr¨¬nh h¨¬nh th¨¤nh b?nh.
  • 9. ? C?n l?m s¨¤ng: + XNM: bc ¨¢i toan t?ng 10% + Test KI: cho ng??i b?nh u?ng KI 1g li?u duy nh?t test ¨¢p t?i ch? m? KI 50% v?i vaselin l¨ºn v¨´ng da b¨¬nh th??ng. ??c test sau 24 ? 48 gi? v¨´ng da th? ng?a, n?i ban ?? ho?c xh b?ng n??c. + MBH: b?ng n??c d??i th??ng b¨¬, ko v¨® hi?n t??ng teo gai, ??nh nh¨² b¨¬ th?m nhi¨ºm BC?N TT
  • 10. ? C?n l?m s¨¤ng: + MDHQ: ti¨ºu chu?n v¨¤ng MDHQTT: l?ng ??ng IGA d?ng H?T ? nh¨² b¨¬ (ranh gi?i gi?a trung b¨¬ v¨¤ h? b¨¬). Ngo¨¤i ra c¨°n th?y C3 v¨¤ Fibrinogen. MDHQGT: 70% IGA l?u h¨¤nh trong m¨¢u
  • 11. ? Ch?n ?o¨¢n x¨¢c ??nh: + Th??ng t?n ?a d?ng: b?ng n??c, m?n n??c, s?n, s?n ph¨´, d¨¢t ?? + B?ng n??c c¨® qu?ng ?? xung quanh, t?p trung th¨¤nh t?ng c?m tr¨ºn n?n d¨¢t ?? + C¨® ti?n tri?u ng¨²a, t¨¢i ph¨¢t, tt b¨¬nh th??ng + Test KI 50% (+) tr¨ºn 85% b?nh nh?n + MBH: b?ng n??c d??i th??ng b¨¬ + MDHQ: l?ng ??ng Ig A d?ng h?t ? nh¨² b¨¬
  • 12. ? Ch?n ?o¨¢n PB: + H?ng ban ?a d?ng + Ch?c + Pemphigus, + Pemphigoid + B?nh zona, + herpes + B?nh ly th??ng b¨¬ b?ng n??c b?m sinh + Ig A th¨¤nh d?i
  • 13. ?i?u tr?: + T?i ch?: ch? y?u d¨´ng dd s¨¢t khu?n t?i ch?: xanhmethylen, millian. N?u b?ng n??c to th¨¬ ch?c v? ?? l?i m¨¤ng v¨¤ ch?m dd sk.TT kh? d¨´ng cort v¨¤ ks b?i. + Thu?c t¨ªm pha 1/10.000 t?m
  • 14. + To¨¤n th?n: - Ch? ?? ?n ko c¨® gluten, h?n ch? ?n iod v¨¤ ?? bi?n - DDS 100-300 mg/ng¨¤y, ?? sau v¨¤i ng¨¤y.D¨´ng duy tr¨¬ ?? h?n ch? t¨¢i ph¨¢t - Sulfapyridin d¨´ng thay th? khi ko ?¨¢p ?ng DDS 1-1,5g/ng¨¤y _ C¨® th? dung cort v?i ks nh¨®m cyclin,
  • 15. Abstract: We report a case of dermatitis herpetiformis co-localised with segmental vitiligo in a 37-year-old woman with a background history of autoimmune polyglandular syndrome type 2. We propose genetic mosaicism as a possible mechanism. There has only been one previous case report in which dermatitis hepetiformis co-localised in close proximity but not exclusively within vilitigo in a patient with autoimmune thyroiditis. To our knowledge, this is the first case report of dermatitis herpetiformis co- localised exclusively to segmental vitiligo in the presence of autoimmune polyglandular syndrome. KEYWORDS: Addison¡¯s disease; Duhring's disease; autoimmune hypothyroidism; autoimmunity; coeliac disease; dermatitis herpetiformis; diabetes mellitus; vitiligo

Editor's Notes

  1. Bullous and vesicular lesions located within segmental vitiligo on left lower arm and a close-up image of representative bullae, vesicles and crusted papules.
  2. Biopsy from the left arm demonstrating subepidermal blister formation (haematoxylin and eosin, original magnification: x200); (B) Direct immunofluorescence with granular IgA deposition in the basement membrane zone at tips of the dermal papillae
  3. Dermatitis herpetiformis: erythematous plaques, grouped vesicles, exulcerations and blood crust in lower limbs
  4. Direct immunofluorescence. Fluorescence in granular pattern in the basement membrane zone