ازﻮﻟﻮﺑ ﺲﻳﺰﻳﻻﻮﻣرﺪﻴﭘا ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد لﺎﺘﻧدﻮﻳﺮﭘ يﺎﻫ ﺖﺒﻗاﺮﻣ
مﺪﻘﻣ ﻲﺷﺎﺑرﺎﻄﻋ ﻪﻠﺿﺎﻓ
،1
مﺪﻘﻣ ﻲﺷﺎﺑرﺎﻄﻋ هﺪﻋﺎﺳ
،2
ﺎﺣ ﺪﻤﺣا ﺮﺋ ﻲﻧﺎﻛدرا نﺎﻳ
،3
يﺪﻤﺣا داﺰﻬﺑ
4
1 . ناﺮﻳا ،دﺰﻳ ،ﻲﻗوﺪﺻ ﺪﻴﻬﺷ ﻲﻜﺷﺰﭘ مﻮﻠﻋ هﺎﮕﺸﻧاد ،ﻲﻜﺷﺰﭙﻧاﺪﻧد هﺪﻜﺸﻧاد ،ﺲﻜﻴﺘﻧدﻮﻳﺮﭘ ﺶﺨﺑ رﺎﻳدﺎﺘﺳا .
2 . ﻮﻟﻮﺗﺎﭘ ﺶﺨﺑ رﺎﻳدﺎﺘﺳا يژ
، ﻲﻜﺷﺰﭙﻧاﺪﻧد هﺪﻜﺸﻧاد
، ﻲﻜﺷﺰﭘ مﻮﻠﻋ هﺎﮕﺸﻧاد ﻲﺘﺸﻬﺑ ﺪﻴﻬﺷ
، ناﺮﻳا ،ناﺮﻬﺗ .
3 . ﺲﻜﻴﺘﻧدﻮﻳﺮﭘ ﺶﺨﺑ رﺎﻴﺸﻧاد
، ﺘﺟا ﻞﻣاﻮﻋ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ ﻮﻀﻋ ناﺪﻧد و نﺎﻫد ﺖﻣﻼﺳ ﺮﺑ ﺮﺛﺆﻣ ﻲﻋﺎﻤ
ﺪﻴﻬﺷ ﻲﻜﺷﺰﭘ مﻮﻠﻋ هﺎﮕﺸﻧاد ،ﻲﻜﺷﺰﭙﻧاﺪﻧد هﺪﻜﺸﻧاد ،
ناﺮﻳا ،دﺰﻳ ،ﻲﻗوﺪﺻ
4 . يﻮﺠﺸﻧاد ﻲﻜﺷﺰﭙﻧاﺪﻧد
، ﺰﭘ مﻮﻠﻋ هﺎﮕﺸﻧاد ﻲﻜﺷ
ﻲﻗوﺪﺻ ﺪﻴﻬﺷ
، دﺰﻳ ناﺮﻳا ، .
ﺖﻓﺎﻳرد ﺦﻳرﺎﺗ :
5 / 3 / 1392
شﺮﻳﺬﭘ ﺦﻳرﺎﺗ :
23 / 4 / 1392
هﺪﻴﻜﭼ ﺲﻳﺰﻳﻻﻮﻣرﺪﻴﭘا ازﻮﻟﻮﺑ
ﻪﺑ ﻲﻫوﺮﮔ زا يرﺎﻤﻴﺑ يﺎﻫ ﻲﻜﻴﺘﻧژ ردﺎﻧ ﻪﺘﻔﮔ ﻲﻣ دﻮﺷ ﻪﻛ ﻪﺼﺨﺸﻣ ﺎﻬﻧآ لوﺎﺗ يﺎﻫ ﻲﺘﺳﻮﭘ و ﻲﻃﺎﺨﻣ ﺖﺳا ﻪﻛ رد
ﻲﻀﻌﺑ دراﻮﻣ رﺎﻜﺳا ﻪﺑ ﺎﺟ ﻲﻣ ﺪﻧراﺬﮔ . رد ﻲﻀﻌﺑ زا عاﻮﻧا ﻦﻳا يرﺎﻤﻴﺑ لوﺎﺗ يﺎﻫ ﺪﻳﺪﺷ ﻪﻛ ﺪﻌﺑ رﺎﻜﺳا ﻪﺑ ﺎﺟ ﻲﻣ ﺪﻨﻧﺎﻣ مﺎﺠﻧا .
شور يﺎﻫ
ﺖﺷاﺪﻬﺑ نﺎﻫد و ناﺪﻧد ياﺮﺑ رﺎﻤﻴﺑ و ﻦﻳﺪﻟاو وا ﻞﻜﺸﻣ ﺖﺳا ﻦﻴﻨﭽﻤﻫ دﺎﺠﻳا
ﺎﻴﻣﻮﺘﺳوﺮﻜﻴﻣ رد
ﺮﺛا ﻦﻳا ﺎﻫرﺎﻜﺳا ياﺮﺑ نﺎﻜﺷﺰﭙﻧاﺪﻧد ﺰﻴﻧ
لﺎﻤﻋا ﻲﻧﺎﻣرد ار ﺎﺑ ﻲﺗﻼﻜﺸﻣ ﻪﺑور
ور ﻲﻣ دزﺎﺳ . زا ﻦﻳا ر و رد ﻦﻳا ﻪﻌﻟﺎﻄﻣ عاﻮﻧا ﻲﻠﺻا ﻦﻳا يرﺎﻤﻴﺑ و ﺖﺒﻗاﺮﻣ يﺎﻫ ﺖﺷاﺪﻬﺑ نﺎﻫد و نﺎﻣرد
يﺎﻫ لﺎﺘﻧدﻮﻳﺮﭘ ﻞﺑﺎﻗ
مﺎﺠﻧا ياﺮﺑ ﻦﻳا نارﺎﻤﻴﺑ روﺮﻣ هﺪﺷ ﺖﺳا .
يﺪﻴﻠﻛ يﺎﻫ هژاو :
،ازﻮﻟﻮﺑ ﺲﻳﺰﻳﻻﻮﻣرﺪﻴﭘا ﻲﺘﺳﻮﭘ يﺎﻫ لوﺎﺗ
ﻲﻃﺎﺨﻣ_
، يﺎﻬﻳرﺎﻤﻴﺑ
،لﺎﺘﻧدﻮﻳﺮﭘ يﺎﻬﺘﺒﻗاﺮﻣ
ﻲﻧﺎﻣرد
) لﻮﺌﺴﻣ هﺪﻨﺴﻳﻮﻧ (
: ﻦﻔﻠﺗ : 09368792807 ﻚﻴﻧوﺮﺘﻜﻟا ﺖﺴﭘ
:
CASE REPORT
ﻪﻣﺪﻘﻣ زﻮﻟﻮﺑ ﺲﻳﺰﻳﻻﻮﻣرﺪﻴﭘا ا
)
epidermolysis bullosa
) ( (EB
ﻪﺑ
قﻼﻃا ﻲﻜﻴﺘﻧژ لﻼﺘﺧا ﻚﻳ زا ﻲﻌﻴﺳو ﻒﻴﻃ ﻪﻛ دﻮﺷ ﻲﻣ
ﺎﺸﻏ و ﺖﺳﻮﭘ ﺺﺨﺸﻣ ﻲﮔﺪﻨﻨﻜﺷ نآ ﻪﺼﺨﺸﻣ ي
ﺖﺳا ﻲﻃﺎﺨﻣ .
ﻲﻣ دﺎﺠﻳا ﺎﻣوﺮﺗ ﻪﺑ ﺦﺳﺎﭘ رد زﻮﻟﻮﺑﻮﻟﻮﻜﻳزو تﺎﻌﻳﺎﺿ يرﺎﻤﻴﺑ ﻦﻳا رد ﺪﻧﻮﺷ . ﻲﺘﻓﺎﺑ فﺎﻜﺷ دﺎﺠﻳا ﻲﺻﺎﺼﺘﺧا ﺢﻄﺳ سﺎﺳا ﺮﺑ يرﺎﻤﻴﺑ ﻦﻳا
نﺪﺷ دراو زا ﺪﻌﺑ ﻪﺑ ﺖﺳﻮﭘ ﻪﺑ ﻲﻜﻴﻧﺎﻜﻣ يﺎﻣوﺮﺗ
هوﺮﮔ ﺮﻳز ﻪﺳ
ﻲﻣ ﻢﻴﺴﻘﺗ ﻲﻠﺻا دﻮﺷ
:
1 . ﻚﻴﺘﻴﻟﻮﻣرﺪﻴﭘا
)
simplex epidermolysis bullosa
(
2 . ﻚﻴﺘﻴﻟوﺪﻴﺳﻮﻟﺎﻨﻴﻣﻻ
)
junctional epidermolysis bullosa
(
3 . ﻚﻴﺘﻴﻟﻮﻣرد
)
dystrophic epidermolysis bullosa
(
ﺪﻨﻋﻮﻨﺘﻣ ﻲﻬﺟﻮﺗ ﻞﺑﺎﻗ رﻮﻃ ﻪﺑ ﻲﺘﺳﻮﭘ تﺎﻌﻳﺎﺿ ﻪﻛ
ﺎﺷ ﻞﻣ
،لوﺎﺗ ﻪﻤﻟد نژورا ﻴﻟﻮﻧاﺮﮔ ﺖﻓﺎﺑ ،رﺎﻜﺳا ،يا
تاﺮﻴﻴﻐﺗ ،ﻦﺸ
نﻮﻴﺳﺎﺘﻧﺎﻤﮕﻴﭘ ﻦﺧﺎﻧ ﻲﻓوﺮﺘﺴﻳد ﺎﻳ ناﺪﻘﻓ و
ﺎﻫ ﺪﻨﺘﺴﻫ . تاﺮﻫﺎﻈﺗ
ناﺪﻧد ،ﺎﻫ ﻢﺸﭼ رد ﺖﺳا ﻦﻜﻤﻣ ﻲﺘﺳﻮﭘ جرﺎﺧ ،نﺎﻫد طﺎﺨﻣ ،ﺎﻫ
يراردا يرﺎﺠﻣ ،ﺪﻌﻘﻣ ،شراﻮﮔ ﻪﻟﻮﻟ ،يﺮﻣ -
ﻢﺘﺴﻴﺳ و ﻲﻠﺳﺎﻨﺗ
ﺪﻧﻮﺷ هﺪﻫﺎﺸﻣ ﻲﺘﻠﻜﺳا ﻲﻧﻼﻀﻋ .
يﺮﻴﮔرد رﺎﺧ يﺎﻫ ﻲﻣ ﻲﺘﺳﻮﭘ ج
ﺷﺎﺑ ﺪﻳﺪﺷ رﺎﻴﺴﺑ ﺪﻧاﻮﺗ ﻨ
ﺪ
، ﻪﺑ ﻪﻛ يﻮﺤﻧ ﻪﺑ ﻪﻈﺣﻼﻣ ﻞﺑﺎﻗ رﻮﻃ
يا
ﺪﻫد راﺮﻗ ﺮﻴﺛﺎﺗ ﺖﺤﺗ ار دﺮﻓ ﻲﮔﺪﻧز ﻚﺒﺳ .
ﺖﻌﺳو و تﺎﻴﺻﻮﺼﺧ
يﺮﻴﮔرد عاﻮﻧا ﻦﻴﺑ ﻲﻧﺎﻫد يﺎﻫ ﺖﺳا عﻮﻨﺘﻣ رﺎﻴﺴﺑEB
. ﻒﻴﻔﺧ رد
مﺮﻓ ﻦﻳﺮﺗ
epidermolysis bullosa
ﻲﺘﺛارو ﺎﻬﻨﺗ نﺎﻫد طﺎﺨﻣ ،
لوﺎﺗ ﻲﻫﺎﮔ ﺎﻬﻟﻮﻜﻳزو ﺎﺑ ﻲﻳﺎﻫ
نﺎﺸﻧ ار ﺮﺸﺘﻨﻣ ﻚﭼﻮﻛ ي ﻲﻣ
ﺪﻫد
ًﺎﻌﻳﺮﺳ ﻪﻛ ﻲﻣ دﻮﺒﻬﺑ رﺎﻜﺳا نوﺪﺑ
ﺮﻴﺛﺎﺗ ﺖﺤﺗ ار دﺮﻓ ﻲﮔﺪﻧز و ﺪﻨﺑﺎﻳ
ﻲﻤﻧ راﺮﻗ ﺪﻨﻫد . نﺎﻫد طﺎﺨﻣ مﺎﻤﺗ ،ﺪﻳﺪﺷ رﺎﻴﺴﺑ دراﻮﻣ رد ﺪﻨﭼﺮﻫ
لوﺎﺗ ﺎﺑ و هﺪﺷ ﺮﻴﮔرد نآ لﺎﺒﻧد ﻪﺑ و ﺪﻳﺪﺷ ﻲﻧﺎﻫد ﻞﺧاد يﺎﻫ
و ﻲﻧﺎﻫد لﻮﺒﻴﺘﺳو نﺪﺷ ﻪﺘﺴﺑ ،ﺎﻴﻣﻮﺘﺳوﺮﻜﻴﻣ و رﺎﻜﺳا ﻞﻴﻜﺸﺗ ﻧا ﺪﺑﺎﻳ ﻲﻣ ﺮﻫﺎﻈﺗ ﺎﺳﻮﻠﮔﻮﻠﻴﻜ )
1 (.
simplex epidermolysis bullosa
رﺎﻤﻴﺑ زا ﻲﻣﺮﻓ ﻪﻛ ﺖﺳا ي
ًﻻﻮﻤﻌﻣ و ﺖﺳاراد ار ﺮﻄﺧ و تﺪﺷ ﻦﻳﺮﺘﻤﻛ ﻪﺑ ﺐﻟﺎﻏ ترﻮﺻ ﻪﺑ
ﻲﻣ ثرا ﺪﺳر . نورد يرﺎﻤﻴﺑ مﺮﻓ ﻦﻳا رد لوﺎﺗ ﻞﻴﻜﺸﺗ
ﺖﻴﺳﻮﻨﻴﺗاﺮﻛ ﻲﻣ قﺎﻔﺗا لازﺎﺑ يﺎﻫ
ﺳﺎﺗﻮﻣ ﺖﻠﻋ ﻪﺑ ﻪﻛ ﺪﺘﻓا رد نﻮﻴ
نژ ﻦﻴﺗاﺮﻛ يﺎﻫ 5
و 14 ﺖﺳا ) 2 ( لوﺎﺗ نارﺎﻤﻴﺑ ﺐﻠﻏا رد .
ﺎﻫ رﺎﻜﺳا يدﻮﺒﻬﺑ زا ﺪﻌﺑ و هدﻮﺑ ﻒﻴﻔﺧ
ﻲﻗﺎﺑ ﻲﻤﻧ ﺪﻧﺎﻣ . عاﻮﻧا ﻲﻀﻌﺑ
ﺮﻳز ﻦﻳا ﺮﻴﮔرد ار ﺎﻫﺎﭘ و ﺎﻬﺘﺳد ﻂﻘﻓ هوﺮﮔ
ﻲﻣ ﺪﻨﻨﻛ ﺪﻨﻧﺎﻣ
"Kallin syndrome"
ﺎﺑ ﻪﻛ ﺖﺳا هاﺮﻤﻫ ﺰﻴﻧ ﺎﻴﺸﻧدﻮﭙﻳﺎﻫ .
عاﻮﻧا
ﺪﻨﺷﺎﺑ ﻪﺘﺷاد يﺮﺗ ﻊﻴﺳو يﺎﻬﻟوﺎﺗ ﺪﻨﻧاﻮﺗ ﻲﻣ هوﺮﮔﺮﻳز ﻦﻳا ﺮﮕﻳد ﺪﻧﻮﺷ ناﺪﻧد و ﻮﻣ نداد ﺖﺳد زا ﺚﻋﺎﺑ ﻪﻛ ﺮﻴﻈﻧ
:
"Dowling
Meara type"
. عﻮﻧ زا هوﺮﮔ ﺮﻳز ﻦﻳا رد ﺎﻬﻟوﺎﺗ
simplex
ازﻮﻟﻮﺑ ﺲﻳﺰﻳﻻﻮﻣرﺪﻴﭘا ﻪﻛ هدﻮﺑ
ًﺎﺗﺪﻤﻋ ﻚﻳژارﻮﻤﻫ )
يﺰﻳﺮﻧﻮﺧ
هﺪﻨﻫد ( ﺑ ﻲﻣ ﺪﻨﺷﺎ ترﻮﺻ ﻪﺑ دﻮﺒﻬﺑ زا ﺲﭘ و
Milia
) ﺖﺴﻴﻛ
ﺪﻨﺘﺴﻫ ﻦﻴﺗاﺮﻛ يﻮﺘﺤﻣ ﻪﻛ ﻲﻧزرا ﻪﻧاد ﻚﭼﻮﻛ يﺎﻫ (
دﻮﺧ زا ﺮﺛا
ﺪﻧراﺬﮔ ﻲﻣ ﻲﻗﺎﺑ )
3 ( ﻟوﺎﺗ دﻮﻋ.
ﻴﻏ ﺎﻬ ﺪﻳﺪﻬﺗ ﺎﻣا ﺖﺳا بﺎﻨﺘﺟا ﻞﺑﺎﻗ ﺮ
ﺖﺴﻴﻧ ﻲﮔﺪﻧز هﺪﻨﻨﻛ )
4
، 1 (.
junctional epidermolysis bullosa
ﻪﺑ بﻮﻠﻐﻣ ترﻮﺻ ﻪﺑ
لوﺎﺗ و ﺪﺳر ﻲﻣ ثرا دﺎﺠﻳا ﻪﻳﺎﭘ ﺎﺸﻏ ياﺪﻴﺳﻮﻟ ﺎﻨﻴﻣﻻ نورد ﺎﻫ
ﻲﻣ و ﺪﻧﻮﺷ آ عﻮﻨﺘﻣ يﺎﻫ ﻲﺘﻴﻟﺎﻣرﻮﻨﺑ ﻲﻤﻫ
دﻮﺟو لﺎﻣوزﻮﻤﺳد
دراد . ﻧﻮﻴﺳﺎﺗﻮﻣ نژ رد يدﺪﻌﺘﻣ يﺎﻬ 3
ﻛ ﻪ 5
anchoring
filament lamin
ﻲﻣ ﺪﻛ ار ﺖﺳا هﺪﺷ شراﺰﮔ ﺪﻨﻛ
. د عﻮﻧ ﻦﻳا ر
زا رﺎﻜﺳا نارﺎﻤﻴﺑ ﺐﻠﻏا ردEB
دﺎﺠﻳا دﻮﺷ ﻲﻤﻧ و
ﺪﻌﺘﺴﻣ ﺖﺳﻮﭘ
ﻧاز و ﺞﻧرآ ﺖﺳﻮﭘ صﻮﺼﺧ ﻪﺑ ﺖﺳا لوﺎﺗ
ًﻻﻮﻤﻌﻣ ﻪﻛ ﻮ ﻲﻣ ﻊﻤﺟ
ﺪﻧﻮﺷ . ﻚﻳ رد زا عﻮﻧ
junctional EB
نآ ﻪﺑ ﻪﻛ
Gravis
junctional EB
)
Herlitz type
( ﻪﺘﻔﮔ دﻮﺷ ﻲﻣ . لوﺎﺗ نآ ﺎﻫ رﺪﻗ
ﻪﺑ دازﻮﻧ ﺖﺳا ﻦﻜﻤﻣ ﻪﻛ ﺪﻨﺘﺴﻫ ﺪﻳﺪﺷ ارﺪﻴﻫد و ﺖﻧﻮﻔﻋ ﺮﻃﺎﺧ
-
هﺪﻧز ﺪﻳﺪﺷ نﻮﻴﺳﺎﺗ ﺪﻧﺎﻤﻧ
) 1،4 .(
dystrophic epidermolysis bullosa
و ﺐﻟﺎﻏ ﻞﻜﺷ ﻪﺑ
ﻲﻣ ثرا ﻪﺑ بﻮﻠﻐﻣ
ًﻻﻮﻤﻌﻣ و ﺪﺳر ﺎﻨﻴﻣﻻ ﺮﻳز حﻮﻄﺳ رد فﺎﻜﺷ ﺎﺑ
اﺪﻴﺳﻮﻟ ) ﺲﻴﻣرد ﻲﺤﻄﺳ يرﻼﻴﭘﺎﭘ رد (
ﻪﺑ ﻪﻛ ﺖﺳا هاﺮﻤﻫ ﺮﻃﺎﺧ
عﻮﻧ نژﻼﻛ نژ رد نﻮﻴﺳﺎﺗﻮﻣ ﻲﻣ VII
ﺪﺷﺎﺑ . ﻧ ﻦﻳا رد زا عﻮ ،EB
ﺪﺣ زا ﺶﻴﺑ ﺰﻴﻟﻮﻧژﻼﻛ نژﻼﻛ ناﺪﻘﻓ ﺎﻳ ﺶﻫﺎﻛ ﻪﺑ ﺮﺠﻨﻣ
ﻪﻛ VII
زوﺮﺒﻴﻓ ﻦﻴﺌﺗوﺮﭘ ﻚﻳ ي
هﺪﻧراﺪﻬﮕﻧ يﺎﻫ ﺖﻨﻣﻼﻴﻓ ﻲﻠﺻا ءاﺰﺟا زا
ﺖﺳا ﻲﻣ ، دﻮﺷ . هﺪﻧراﺪﻬﮕﻧ يﺎﻫ ﻞﻳﺮﺒﻴﻓ
، ﺶﻫﺎﻛ ﺎﻳ ﻲﻌﻴﺒﻃ ﺮﻴﻏ
ﺪﻨﺘﺴﻫ ﻪﺘﻓﺎﻳ و
ًﻼﻛ ﺎﻳ زا لﺎﻣرﺪﻴﭘا لﺎﺼﺗا و ﺪﻧراﺪﻧ دﻮﺟو ﻦﻴﺑ
ﺖﺳا ﻪﺘﻓر .
ﻚﻴﻓوﺮﺘﺴﻳد عﻮﻧ
،EB
ﻲﻣ دﺎﺠﻳا ﻲﻳﺎﻬﻟوﺎﺗ ﺪﻨﻛ
ﻪﻛ
ﺎﻴﺴﺑ نآ تﺪﺷ لﻮﻤﻌﻣ رﻮﻃ ﻪﺑ ﺎﻣا ﺖﺳا عﻮﻨﺘﻣ ر
ﺖﺳد ﺎﭘ و ﺎﻫ ﺎﻫ
ﻲﻣ ﺮﻴﮔرد ار ﺪﻨﻛ
. ﻪﻛ نآ عﻮﻧ ﻚﻳ رد
Hallopeau-Siemens EB
دراد مﺎﻧ
، لوﺎﺗ ﺎﻫ لﺎﺼﺗا ﻪﺑ ﺮﺠﻨﻣ ﺎﭘ و ﺖﺳد رﺎﻜﺳا و رﺮﻜﻣ ي
ﺚﻋﺎﺑ و هﺪﺷ ﺎﭘ و ﺖﺳد نﺎﺘﺸﮕﻧا دﺎﺠﻳا
ﻪﻴﺒﺷ ﻲﻳﺎﻤﻧ ﻪﺑ
ﺶﻜﺘﺳد
و
ًﺎﺘﻳﺎﻬﻧ دﻮﺷ ﻲﻣ نﺎﺘﺸﮕﻧا دﺮﻜﻠﻤﻋ ناﺪﻘﻓ .
ﺪﻟﻮﺗ نﺎﻣز رد يرﺎﻤﻴﺑ
ﻲﻣ هﺪﻫﺎﺸﻣ نآ زا ﺪﻌﺑ ﻲﻤﻛ ﺎﻳ دﻮﺷ
) 5
، 4
، 1 (.
ﻒﻠﺘﺨﻣ يﺎﻬﻧژ رﺎﻤﺷ ﻲﺑ و دﺪﻌﺘﻣ يﺎﻬﻧﻮﻴﺳﺎﺗﻮﻣ ﻲﻠﻛ رﻮﻃ ﻪﺑ زوﺮﺑ رد ﻞﻴﺧد ي هﺪﺷ ﻪﺘﺧﺎﻨﺷ عﻮﻨﺘﻣ يﺎﻫ ﭗﻴﺗﻮﻨﻓ و EB
ﻪﻛ ﻲﺗﺎﻌﻟﺎﻄﻣ زا ﻪﺘﺳد نآ ياﺮﺑ ار يﺮﻴﮔ ﻪﺠﻴﺘﻧ ،نآ زا ﻞﺻﺎﺣ ﭗﻴﺗﻮﻨﻓ - ﭗﻴﺗﻮﻧژ
ًﺎﺒﻗﺎﻌﺘﻣ و يﺎﻬﻫوﺮﮔﺮﻳز رد ار ﻲﻠﻟآ يﺎﻬﻧﻮﻴﺳﺎﺗﻮﻣ
راﺮﻗ ﻲﺳرﺮﺑ درﻮﻣ EB
هدﺮﻛ ﻲﮔﺪﻴﭽﻴﭘ رﺎﭼد ،ﺪﻧا هداد
ﺖﺳا ) 6،7 .(
و تﺎﻌﻳﺎﺿ ﻲﻛﺎﻧدرد ﻟوﺎﺗ
ﺎﻬ ﺖﻳدوﺪﺤﻣ ، رد
زﺎﺑ ندﺮﻛ نﺎﻫد و
ﻲﻧاﻮﺗﺎﻧ رد هدﺎﻔﺘﺳا زا ﺎﻬﺘﺳد و نﺎﺘﺸﮕﻧا ﻪﺑ رﻮﻈﻨﻣ كاﻮﺴﻣ
،ندز
زا ﻪﻠﻤﺟ ﻲﻠﻣاﻮﻋ ﺪﻨﺘﺴﻫ ﻪﻛ رد طﺎﺒﺗرا ﺎﺑ ﺸﻧدﻮﻳﺮﭘ ﻢﻴ نﺎﻳﻼﺘﺒﻣ ﻪﺑ
،EB
ﻆﻔﺣ ﺖﺷاﺪﻬﺑ ﻲﻧﺎﻫد ار رد ﻦﻳا داﺮﻓا ﺎﺑ هﺮﻃﺎﺨﻣ ﻪﺟاﻮﻣ ﻲﻣ
دزﺎﺳ ) 8 (.
ﺖﻴﻤﻫا ﻦﻳا عﻮﺿﻮﻣ زا نآ ﺖﻬﺟ تﺎﻌﻟﺎﻄﻣ ﺐﻟﺎﻏ ﻪﻛ
ﻢﺸﭼ ﺎﻫ و طﺎﺨﻣ نﺎﻫد ﻦﻳﺮﺘﻌﻳﺎﺷ ار يﺎﻬﻠﺤﻣ
ﻼﺘﺑا ﻪﺑ تﺎﻌﻳﺎﺿ
ﻲﻃﺎﺨﻣ
،ﺪﻧا هدﻮﻤﻧ شراﺰﮔ EB
ود ناﺪﻨﭼ ﻲﻣ دﻮﺷ ) 12 - 9 .(
ﻦﻴﻨﭽﻤﻫ ضراﻮﻋ
ﻲﻃﺎﺨﻣ - ﻲﻧﺎﻫد ﻲﺷﺎﻧ زا ﻼﺘﺑا ﻪﺑ ﺎﻬﻨﺗ EB
ﻲﻠﻳﻻد ﺪﻨﺘﺴﻴﻧ ﻪﻛ ﻲﻳاﺮﭼ ﻲﺳرﺮﺑ ﻦﻳا يرﺎﻤﻴﺑ ار زا هﺎﮔﺪﻳد
ﺘﻧدﻮﻳﺮﭘ لﺎ ﻪﻴﺟﻮﺗ ﻲﻣ ﺪﻨﻛ ﻪﻜﻠﺑ داﺪﻌﺘﺳا هﻮﻘﻟﺎﺑ ي يﻼﺘﺑا ﻦﻳا
نارﺎﻤﻴﺑ ﻪﺑ تﺎﻌﻳﺎﺿ ﻊﻴﺳو و كﺎﻧدرد ﻲﺷﺎﻧ زا يﺎﻣوﺮﺗ ﻲﻜﻴﻧﺎﻜﻣ
هدراو رد نﺎﻳﺮﺟ ﺖﻴﻟﺎﻌﻓ يﺎﻫ يدﺎﻋ ﻲﮔﺪﻧز ﺮﻴﻈﻧ كاﻮﺴﻣ ندز و
ﻲﺘﺣ فﺮﺼﻣ يﺎﻫاﺬﻏ ﺮﻴﻏ مﺮﻧ و ...
زا ﻪﻠﻤﺟ ﻲﻠﻠﻋ ﺖﺳا ﻪﻛ ﺎﻣ ار
ﺮﺑ نآ ﻲﻣ دراد ﺎﺗ ﻪﺑ عﻮﺿﻮﻣ يﺎﻬﺘﺒﻗاﺮﻣ ﺘﻧدﻮﻳﺮﭘ
لﺎ رد نﺎﻳﻼﺘﺒﻣ
ﻪﺑ ﻢﻳزادﺮﭙﺑ EB
) 13 (.
ﻲﺗﻼﻜﺸﻣ رﺎﻨﻛ رد هﺪﺷ ﺮﻛذ ﻞﻣاﻮﻋ
ﻲﻜﺷﺰﭙﻧاﺪﻧد لﺎﻤﻋا ﻦﻴﺣ ﺪﺣ زا ﺶﻴﺑ يﺰﻳﺮﻧﻮﺧ نﻮﭽﻤﻫ
) ﻲﻤﺟﺎﻬﺗ لﺎﻤﻋا ﺎﺻﻮﺼﺨﻣ (
ﻪﺑ نﻮﺧ نداد ﺖﺳد زا ﻲﺷﺎﻧ ﻲﻤﻧآ و
ﻲﻧﺎﻫد تﺎﻌﻳﺎﺿ و ﺎﻬﻤﺧز ي ﻪﻄﺳاو هﺮﻣز رد ﺰﻴﻧ نﺎﻳﻼﺘﺒﻣ ردEB
ﺮﻄﻣ تﻼﻜﺸﻣ ي دﺮﻴﮔ ﻲﻣ راﺮﻗ نارﺎﻤﻴﺑ ﻦﻳا ياﺮﺑ ح
) 14 (.
ﻲﻧﺎﻫد يﺎﻫ ﻪﺘﻓﺎﻳ
ﺖﻓﺎﺑ تﺎﻌﻳﺎﺿ دﺎﺠﻳا نآ لﺎﺒﻧد ﻪﺑ و نﺎﻫد طﺎﺨﻣ ﻲﮔﺪﻨﻨﻜﺷ ﻲﻠﺻا عاﻮﻧا مﺎﻤﺗ رد نﺎﻫد ﻞﺧاد مﺮﻧ ﺖﺳا ﻊﻳﺎﺷEB
. نارﺎﻤﻴﺑ رد
ﻪﺑ ﻼﺘﺒﻣ
Simplex EB
ﻬﻟوﺎﺗ ، ﻲﻣ دﺎﺠﻳا ﻲﻧﺎﻫد ﻞﺧاد يﺎ دﻮﺷ
. ﻦﻳا
ﻚﭼﻮﻛ تﺎﻌﻳﺎﺿ ﻻﻮﻤﻌﻣ
زا ﺮﺘﻤﻛ 1 و هدﻮﺑ ﺮﺘﻣ ﻲﺘﻧﺎﺳ
ًﻻﻮﻤﻌﻣ
دﻮﺒﻬﺑ رﺎﻜﺳا نوﺪﺑ ﻲﻣ
ﻳ ﺪﻨﺑﺎ . ﻲﻣ ﺮﻈﻧ ﻪﺑ ﻪﻜﻨﻳا ﺎﺑ يﺮﻴﮔرد ﺪﺳر
رد ﻲﻧﺎﻫد ﻞﺧاد
Simplex EB
رد يدازﻮﻧ نارود ﺖﺳا ﺮﺗ ﻊﻳﺎﺷ
، ﻪﻣادا داﺮﻓا ﻲﻀﻌﺑ ﺎﻣا ي
لوﺎﺗ ار ﺎﻫ يدازﻮﻧ زا ﺪﻌﺑ ﻲﺘﺣ و
ﺎﺗ ﺮﺧاوا
ﺪﻨﻨﻛ ﻲﻣ ﻪﺑﺮﺠﺗ ﺰﻴﻧ ﻲﻛدﻮﻛ .
ا ﺮﺜﻛ داﺮﻓا ي ﻪﻛ ثراﻮﺗ يﻮﮕﻟا ﺎﺑ
ﺐﻟﺎﻏ عاﻮﻧا ﻪﺑ ﻼﺘﺒﻣ
Junctional
و
Dystrophic
ﺪﻴﭘا ﺲﻳﺰﻳﻻﻮﻣر
ﻲﺘﺛارو يازﻮﻟﻮﺑ ﺪﻨﺘﺴﻫ
، ياراد ﻲﻧﺎﻫد ﻞﺧاد تﺎﻌﻳﺎﺿ ﺪﻨﺷﺎﺑ ﻲﻣ
رﻮﻃ ﻪﺑ ﻪﻛ زا ﺮﺘﮔرﺰﺑ ﺺﺨﺸﻣ
1 هدﻮﺑ ﺮﺘﻣ ﻲﺘﻧﺎﺳ
، نﺎﺷداﺪﻌﺗ
عﻮﻧ زا ﺮﺘﺸﻴﺑ
Simplex EB
،
ًﻻﻮﻤﻌﻣ و ﻮﻳزورا ﺐﻠﻏا كﺎﻧدرد
ﺪﻨﺘﺴﻫ ) 1،15 .(
يﺎﻣﻮﻨﻴﺳرﺎﻛ ﻞﺳ سﻮﻣاﻮﻜﺳا ﻪﺑ ﻼﺘﺑا ﻚﺴﻳر
رد ﻲﻧﺎﻫد داﺮﻓا
ﻪﺑ ﻼﺘﺒﻣ
ًﺎﺻﻮﺼﺨﻣ و EB
عﻮﻧ بﻮﻠﻐﻣ مﺮﻓ
Dystrophic
ناﺮﮕﻳد ﻪﺑ ﺖﺒﺴﻧ ، ﻪﺑ
ﻃ ﻲﺼﺨﺸﻣ رﻮ ﺮﺗﻻﺎﺑ
اﺰﮔ ر ش
ﺖﺳا هﺪﺷ .
ترﻮﺻ ﻪﺑ ﺖﺴﻳﺎﺑ ﻲﻣ نﺎﻳﻼﺘﺒﻣ زا ﻪﺘﺳد ﻦﻳا ور ﻦﻳا زا
ناﺰﻴﻣ ﺮﻴﻈﻧ ﻲﻧﺎﻫد يﺎﻬﻤﺧز رد تاﺮﻴﻴﻐﺗ ظﺎﺤﻟ زا يا هرود ﻪﻨﻳﺎﻌﻣ درﻮﻣ ،ﻢﺧز ي ﻪﻴﺷﺎﺣ رد هﺪﻋﺎﻗ ﻲﺘﻔﺳ ﺎﻳ و ﻢﺧز شﺮﺘﺴﮔ ﺪﻧﺮﻴﮔ راﺮﻗ )
19 - 16 .(
يﺎﻨﻴﻣ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد ﻲﻧاﺪﻧد
ًﻻﻮﻤﻌﻣ EB
ﺺﻘﻧ رﺎﭼد
هدﻮﺑ ﻞﻜﺷ ﻚﻴﺘﺳﻼﭘﻮﭙﻳﺎﻫ ﺎﻳ رﺎﻴﺷ ياراد و
ﺪﺷﺎﺑ ﻲﻣ . زا ﻲﺧﺮﺑ
ﺎﺑ ﻂﺒﺗﺮﻣ يﺎﻫ ﻦﻴﺌﺗوﺮﭘ ﻦﻴﻨﻴﻣﻻ ﺮﻴﻈﻧ EB
332 ﻞﻴﻜﺸﺗ ياﺮﺑ
ﻪﺠﻴﺘﻧ رد ﺎﻬﻨﻴﺌﺗوﺮﭘ ﻦﻳا دﻮﺒﻤﻛ و ﺪﻨﺘﺴﻫ يروﺮﺿ ﻲﻌﻴﺒﻃ يﺎﻨﻴﻣ ﻪﺑ ﻼﺘﺑا ي ﻲﻔﻠﺘﺨﻣ تﺎﺟرد ترﻮﺻ ﻪﺑ نﺎﻳﻼﺘﺒﻣ رد ،EB
زا
ﺪﻳﺎﻤﻧ ﻲﻣ زوﺮﺑ ﻲﻳﺎﻨﻴﻣ يزﻼﭘﻮﭙﻳﺎﻫ .
ﻲﻃ ﻪﻛ يﺎﻬﻨﻴﺌﺗوﺮﭘ ﺮﮕﻳد زا
ﻪﺑ ﻼﺘﺑا ﺺﻘﻧ رﺎﭼد EB
ددﺮﮔ ﻲﻣ ﻦﻴﻟﺪﻨﻴﻛ ناﻮﺗ ﻲﻣ ، -
1 مﺎﻧ ار
طﺎﺨﻣ مﺎﺠﺴﻧا ﻆﻔﺣ رد ﻪﻛ دﺮﺑ
، ﺪﻨﻛ ﻲﻣ ﺎﻔﻳا ﺶﻘﻧ رد ﺎﻣا
دراﺪﻧ ﻲﺘﻟﺎﺧد ناﺪﻧد ﻞﻴﻜﺸﺗ )
20 (.
ﺪﺷ ﻪﺘﻔﮔ ﻪﻛ رﻮﻄﻧﺎﻤﻫ
، رﺎﻜﺳا ﻊﻴﺳو ﻲﻧﺎﻫد ﺪﻧاﻮﺗ ﻲﻣ ﺚﻋﺎﺑ
نﺎﺑز ﺖﻛﺮﺣ و هﺪﺷ ﺎﻴﺳﻮﻠﮔﻮﻠﻴﻜﻧا و ﺎﻴﻣﻮﺘﺳوﺮﻜﻴﻣ ﻦﻳا رد ار
نارﺎﻤﻴﺑ دوﺪﺤﻣ ﺪﻳﺎﻤﻧ . نارﺎﻤﻴﺑ ﻦﻳا رد
، زا لﺎﺗﻻﺎﭘ يﺎﮔور ﻦﻴﺑ
ﻪﺘﻓر و ﻖﻤﻋ لﻮﺒﻴﺘﺳو ﺶﻫﺎﻛ
ﺪﺑﺎﻳ ﻲﻣ . مﺮﻧ ﺖﻓﺎﺑ تﺎﻌﻳﺎﺿ دﻮﺟو
ًﺎﺻﻮﺼﺨﻣ ﻪﺑ ﺮﺠﻨﻣ ﺪﻨﺘﺴﻫ ﺪﻳﺪﺷ تﺎﻌﻳﺎﺿ ﻦﻳا ﻪﻛ يدراﻮﻣ رد
اﺬﻏ ﻢﻳژر زا هدﺎﻔﺘﺳا دﻮﺷ ﻲﻣ تارﺪﻴﻫﻮﺑﺮﻛﺮﭘ ﺐﻠﻏا و مﺮﻧ ﻲﻳ
. ﻪﺑ
ﻛﺮﺣ رد ﺖﻳدوﺪﺤﻣ ﺖﻠﻋ ﺎ
لﻮﺒﻴﺘﺳو ﻖﻤﻋ ﺶﻫﺎﻛ و نﺎﺑز ت
، نﺎﻣز
ﺪﺑﺎﻳ ﻲﻣ ﺶﻳاﺰﻓا نﺎﻫد زا ﻲﻳاﺬﻏ داﻮﻣ نﺪﺷ كﺎﭘ .
هوﻼﻋ ﻪﺑ
ﻒﻴﻌﻀﺗ نﺎﺘﺸﮕﻧا ﻲﻠﻜﺷﺪﺑ هاﺮﻤﻫ ﻪﺑ نﺎﻫد مﺮﻧ و ﺖﺨﺳ يﺎﻬﺘﻓﺎﺑ
ناﺪﻧد و نﺎﻫد ﺖﺷاﺪﻬﺑ ﺖﻳﺎﻋر رد ﻲﻧاﻮﺗﺎﻧ ﻪﺑ ﺮﺠﻨﻣ ﻲﻣ رﺎﻤﻴﺑ رد
ددﺮﮔ ﺮﺑ ﻲﻠﻴﻟد دﻮﺧ ﻪﻛ ﺑ عﻮﻴﺷ
ﺮﺘﺸﻴ يرﺎﻤﻴﺑ و ﻲﮔﺪﻴﺳﻮﭘ
داﺮﻓا ﻦﻳا رد لﺎﺘﻧدﻮﻳﺮﭘ ﺖﺳا
) 23 - 21 .(
لﺎﺘﻧدﻮﻳﺮﭘ يﺎﻬﺘﺒﻗاﺮﻣ
نﺎﻫد ﺖﺷاﺪﻬﺑ :
و كاﻮﺴﻣ زا هدﺎﻔﺘﺳا درﻮﻣ رد ﻲﻧاﺮﮕﻧ
نﺎﻫد طﺎﺨﻣ ﻪﺑ ﺐﻴﺳآ ﻞﻴﺴﻧﺎﺘﭘ ﺎﻬﻨﺗ
ﻪﺑ فﻮﻄﻌﻣ ﻦﻳا
نارﺎﻤﻴﺑ
و هدﻮﺒﻧ نﺎﻜﺷﺰﭙﻧاﺪﻧد و ﻦﻳﺪﻟاو دﻮﺷ ﻲﻣ ﻞﻣﺎﺷ ﺰﻴﻧ ار
) 24 (.
مﺎﺠﻧا
ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ مﺎﻤﺗ ياﺮﺑ كاﻮﺴﻣ وEB
ﻼﺘﺒﻣ ﻲﺘﺣ نﺎﻳ
ﻪﺑ مﺮﻓ
ﺪﻳﺪﺷ
Dystrophic EB
ﺖﺳا ﺮﻳﺬﭘ نﺎﻜﻣا .
ﻲﻣ ﺮﻳز تادﺎﻬﻨﺸﻴﭘ
ﺪﺷﺎﺑ هﺪﻨﻨﻛ ﻚﻤﻛ ﺐﺳﺎﻨﻣ كاﻮﺴﻣ بﺎﺨﺘﻧا ياﺮﺑ ﺪﻧاﻮﺗ :
ﺪﺷﺎﺑ ﻚﭼﻮﻛ كاﻮﺴﻣ ﺮﺳ .
ﺪﺷﺎﺑ مﺮﻧ كاﻮﺴﻣ يﺎﻫزﺮﭘ .
كاﻮﺴﻣ زا ﻞﺒﻗ مﺮﮔ بآ ﺎﺑ كاﻮﺴﻣ يﻮﺸﺘﺴﺷ ﺎﺑ ناﻮﺗ ﻲﻣ ندز مﺮﻧ اﺮﻧآ دﺮﻛ ﺰﻴﻧ ﺮﺗ .
ناﻮﺗ ﻲﻣ ﺪﻳﺪﺷ يﺎﻴﻣﻮﺘﺳوﺮﻜﻴﻣ ﺎﺑ نارﺎﻤﻴﺑ رد ﺎﻫزﺮﭘ
هﺎﺗﻮﻛ ار
اﺪﻧد لازﻮﻠﻛا ﺢﻄﺳ ناﻮﺘﺑ ﺮﺘﻬﺑ ﺎﺗ دﺮﻛ دﺮﻛ ﺰﻴﻤﺗ ار ﺎﻬﻧ
. ﺪﻳﺎﺑ
ﺎﻫزﺮﭘ ﺪﺷ ﻦﺌﻤﻄﻣ ﻲﻗﺎﺑ مﺮﻧ زﻮﻨﻫ نﺪﺷ هﺎﺗﻮﻛ زا ﺪﻌﺑ
ﻲﻣ
ﺪﻨﻧﺎﻣ ) 25 (.
بآ ﺎﺑ نﺎﻫد يﻮﺸﺘﺴﺷ هﺪﻋو زا ﺪﻌﺑ صﻮﺼﺧ ﻪﺑ زور لﻮﻃ رد
ﻪﺑ ﻲﻳاﺬﻏ يﺎﻫ ﻆﻔﺣ
ﻚﻤﻛ نﺎﻫد ﺖﺷاﺪﻬﺑ ﺪﻨﻛ ﻲﻣ
ﻪﻛ اﺮﭼ
ﺖﺷادﺮﺑ ﺚﻋﺎﺑ تﺎﺑﻮﺳر و ﺎﻫ يﺮﺑد
ﻲﻳاﺬﻏ داﻮﻣ دﻮﺷ ﻲﻣ
) 25 (.
ﻞﻤﻜﻣ ﻦﻳﺪﻳﺰﮕﻫﺮﻠﻛ ﻪﻳﻮﺸﻧﺎﻫد ﺪﻨﻧﺎﻣ نﺎﻫد ﺖﺷاﺪﻬﺑ يﺎﻫ
12 / 0 % رد نﺎﻫد يﺎﻬﻳرﺎﻤﻴﺑ زا يﺮﻴﮕﺸﻴﭘ ياﺮﺑ ﻲﻌﻴﺳو رﻮﻃ ﻪﺑ
ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ ﻲﻣ ﻪﻴﺻﻮﺗ EB
دﻮﺷ
، ﻪﻛ ﺪﻨﭼﺮﻫ لﺮﺘﻨﻛ رد
ﻳا رد ﻪﻛ ﺖﺳا ﺪﻣآرﺎﻛﺎﻧ ﻲﮔﺪﻴﺳﻮﭘ ﻪﻳﻮﺸﻧﺎﻫد زا ﺪﻳﺎﺑ درﻮﻣ ﻦ
ﺪﻳارﻮﻠﻓ دﻮﺷ هدﺎﻔﺘﺳا .
شور زا ﻲﻜﻳ ﻫﺮﻠﻛ زا هدﺎﻔﺘﺳا يﺎﻫ
ﻦﻳﺪﻳﺰﮕ
ياﺮﺑ زور رد رﺎﺑ ود ندﺮﻛ ﻪﻳﻮﺸﻧﺎﻫد نارﺎﻤﻴﺑ ﻦﻳا رد 2
ﺮﻫ و ﻪﺘﻔﻫ
ﻚﻳ هﺎﻣ ﻪﺳ ﺖﺳا رﺎﺑ
. رد ﻪﻳﻮﺸﻧﺎﻫد ﻦﻳا ﻞﻜﻟا نوﺪﺑ نﻮﻴﺳﻻﻮﻣﺮﻓ
ﻲﻣ ﻪﻴﺻﻮﺗ ﻲﻧﺎﻫد تﺎﻌﻳﺎﺿ ياراد داﺮﻓا ددﺮﮔ
) 25،26 .(
لﺎﺘﻧدﻮﻳﺮﭘ يﺎﻬﻧﺎﻣرد
ي هدﺎﻔﺘﺳا زا ﺪﻨﻤﺘﻗد
ﻚﻴﻨﻜﺗ و ﻚﻴﻧﻮﺳاﺮﺘﻟوا يﺎﻫﺮﻠﻴﻜﺳا
د ندﺮﻛ ﺶﻴﻟﺎﭘ يﺎﻫ ر
ﺪﺷﺎﺑ ﺪﻴﻔﻣ ﺪﻧاﻮﺗ ﻲﻣ نارﺎﻤﻴﺑ مﺎﻤﺗ )
25 (.
ﻗ ﺒﻟ ﻲﻜﺷﺰﭙﻧاﺪﻧد يﺎﻫرﺎﻛ عوﺮﺷ زا ﻞﺒ ﺮﮕﻳد ﺎﻳ ﻦﻴﻟزاو ﺎﺑ ﺪﻳﺎﺑ ﺎﻬ
ﻮﺷ بﺮﭼ داﻮﻣ ﻧ
ﺎﻳ ﻞﻳﺎﺳو نﺪﻴﺒﺴﭼ زا ﺎﺗ ﺪ ﻲﺸﺸﻛ يﺎﻫوﺮﻴﻧ دﺎﺠﻳا
ﺖﻌﻧﺎﻤﻣ ،دﻮﺷ ﻲﻣ تﺎﻌﻳﺎﺿ ﻞﻴﻜﺸﺗ و ﺎﻫ ﺖﻓﺎﺑ ﻪﺑ ﺐﻴﺳآ ﺚﻋﺎﺑ ﻪﻛ ددﺮﮔ ) 25،27 .(
ور ﻦﻳا زا
هﺪﺷ ﻪﻴﺻﻮﺗ ﺰﻴﻧ هدﺎﻔﺘﺳا درﻮﻣ ﻞﻳﺎﺳو ﻦﺸﻴﻜﻳﺮﺑﻮﻟ
ﺖﺳا
، ﺪﻨﭼﺮﻫ ﺎﻛ مﺎﺠﻧا ﻪﻛ ﺮﺗ ﻞﻜﺸﻣ ار ﻲﻜﺷﺰﭙﻧاﺪﻧد يﺎﻫر
ﻪﺘﺧﺎﺳ
و رﺎﻛ ﻦﻳا ﻪﻛ ﺪﻧراد دﺎﻘﺘﻋا يا هﺪﻋ دﻮﺳ
رﺎﻤﻴﺑ ياﺮﺑ ﻲﻧاﺪﻨﭼ
دراﺪﻧ . بآ رد لﻮﻠﺤﻣ يﺎﻫ ﺖﻨﻜﻳﺮﺑﻮﻟ زا ﺪﻳﺎﺑ ﻞﻤﻋ قﺎﺗا رد ﻪﺑ
ندﻮﺑ لﺎﻌﺘﺷا ﻞﺑﺎﻗ ﺮﻴﻏ ﻞﻴﻟد دﻮﻤﻧ هدﺎﻔﺘﺳا
) 25 (.
ندز رﺎﻨﻛ ياﺮﺑ
ًﺎﺤﻴﺟﺮﺗ ﺎﻫ ﻪﻧﻮﮔ و ﺐﻟ ﻪﻛ دﺮﻛ هدﺎﻔﺘﺳا ﻦﻬﭘ ﻞﻳﺎﺳو زا ﺪﻳﺎﺑ
ﺶﻫﺎﻛ ار ﺎﻫ ﺖﻓﺎﺑ ﺮﺑ هدراو يوﺮﻴﻧ ﺪﻫد
) 27 (.
،ﻦﺸﻛﺎﺳ درﻮﻣ رد
ﺎﻫ ناﺪﻧد لازﻮﻠﻛا ﺢﻄﺳ ﻞﺜﻣ ﺖﺨﺳ يﺎﻫ ﺖﻓﺎﺑ يور ﺪﻳﺎﺑ
، يور ﺎﻳ
ﻪﺒﻨﭘ لور ﻚﻳ ﺪﻧﻮﺷ هداد راﺮﻗ
زا و زا هدﺎﻔﺘﺳا يﻮﻗ يﺎﻫ ﻦﺸﻛﺎﺳ
)
high vacuum
( دﻮﺷ بﺎﻨﺘﺟا )
25 (.
زا يﺮﻴﮔ هﺮﻬﺑ يﺎﻫ ﻦﺸﻛﺎﺳ
ياﺮﺑ ﻚﭼﻮﻛ و دﺮﮔ ﻲﻜﻴﺘﺳﻼﭘ ﺮﺳ ﺎﺑ ﻲﻧﺎﻫد يﺎﻫ ﻲﺣاﺮﺟ
درﻮﻣ
ﻣ ﺪﻴﻛﺎﺗ ﺪﺷﺎﺑ ﻲ ) 27 (.
مﺎﺠﻧا لﺎﺘﻧدﻮﻳﺮﭘ ﻲﺣاﺮﺟ ياﺮﺑ
ﻪﺑ نﺎﻳﻼﺘﺒﻣ مﺎﺠﻧا ﻞﺑﺎﻗ EB
ﺖﺳا ) 28 (.
ﻲﻠﻳﻻد ﻪﺑ ﺎﻨﺑ ﻪﻛ دﻮﺷ ﻲﻣ ناﻮﻨﻋ ﻲﻟﺎﺣ رد ﻪﺘﻔﮔ ﻦﻳا
ﺪﺣ زا ﺶﻴﺑ يﺰﻳﺮﻧﻮﺧ نﻮﭽﻤﻫ ﻞﻤﻋ ﻦﻴﺣ
، ﺘﺒﻗاﺮﻣ يﺎﻬ صﺎﺧ ﻪﺑ
رﻮﻈﻨﻣ ﻲﺣاﺮﺟ نارﺎﻤﻴﺑ ﻪﺑ ﻼﺘﺒﻣ
Dystrophic EB
رد ﺖﺴﻳﺎﺑ ﻲﻣ
دﻮﺷ ﻪﺘﻓﺮﮔ ﺮﻈﻧ .
ﺣاﺮﺟ مﺎﺠﻧا ﻲ
ﻲﺘﺳﻼﭘﻮﻟﻮﺒﻴﺘﺳو نﺎﻳﻼﺘﺒﻣ ياﺮﺑ
ﺎﻬﻨﺗ ﻲﻧﺎﻣز دﻮﺷ مﺎﺠﻧا ﺖﺴﻳﺎﺑ ﻲﻣ ﻦﺸﻜﻧﺎﻓ ﺎﻳ ﻲﮔﺪﻧز ﺖﻴﻔﻴﻛ ﻪﻛ
ﻲﻧﺎﻫد ﺪﺷﺎﺑ هﺪﻳدﺮﮔ ﺮﺛﺎﺘﻣ رﺎﻤﻴﺑ .
ﺎﺑ ياﺮﺑ ﻧاﺪﻧد يزﺎﺳز ﺎﻬ
،
دراد ﺖﻴﺤﺟرا ﺖﺑﺎﺛ يﺎﻫﺰﺗوﺮﭘ .
،ﻦﻳا ﺮﺑ ﻖﺑﺎﺳ ﺖﻨﻠﭙﻤﻳا زا هدﺎﻔﺘﺳا
ﻦﻳا رد ﻲﻧاﻮﺨﺘﺳا يﺎﻫﺪﻧﻮﻴﭘ نداد راﺮﻗ و ﻲﻧاﺪﻧد يﺎﻫ ﺎﺑ نارﺎﻤﻴﺑ
ﺖﺳا هﺪﺷ مﺎﺠﻧا ﺖﻴﻘﻓﻮﻣ )
31 - 25 .(
يﺮﻴﮔ ﻪﺠﻴﺘﻧ
ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ ﻪﻜﻨﻳا ﺎﺑ و ﻲﻧﺎﻫد ﻂﻳاﺮﺷ ﺮﻈﻧ زا EB
نﺎﻜﺷﺰﭙﻧاﺪﻧد ياﺮﺑ رﺎﻛ ﻦﻴﺣ تﻼﻜﺸﻣ ﻪﻠﻤﺟ زا
و ﺖﺨﺳ دراﻮﻣ
دﺮﻓ ﻪﺑ ﺮﺼﺤﻨﻣ ﺪﻨﻳآ ﻲﻣ رﺎﻤﺷ ﻪﺑ
، ﺎﻣا دﻮﺟﻮﻣ تﻻﺎﻘﻣ و تﺎﻋﻼﻃا
ﻪﻛ ﺖﺳا ﺐﻠﻄﻣ ﻦﻳا هﺪﻨﻫد نﺎﺸﻧ ﺎﺑ ناﻮﺗ ﻲﻣ
ﻦﺘﻓﺮﮔ ﺮﻈﻧ رد
ﻂﻳاﺮﺷ ﺎﻬﻧآ ﻪﺑ صﻮﺼﺨﻣ ﺖﺷاﺪﻬﺑ شزﻮﻣآ و داﺮﻓا ﻦﻳا صﺎﺧ
، زا
ﻲﺑﻮﻠﻄﻣ ﺪﺣ ﺎﺗ لﺎﺘﻧدﻮﻳﺮﭘ يﺎﻫ يرﺎﻤﻴﺑ و ﻲﮔﺪﻴﺳﻮﭘ دﺎﺠﻳا نﺎﻣرد ﻪﺑ زﺎﻴﻧ ﻪﻛ ﻲﺗرﻮﺻ رد و دﺮﻛ يﺮﻴﮔﻮﻠﺟ
،ﺪﺷﺎﺑ يﺎﻬﻧﺎﻣرد
داد مﺎﺠﻧا نﺎﺸﻳاﺮﺑ صﺎﺧ تﺎﻜﻧ ﺖﻳﺎﻋر ﺎﺑ ار لﻮﻤﻌﻣ .
Refrences
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7. Varki R, Sadowski S, Uitto J, Pfendner E. Epidermolysis bullosa. II. Type VII collagen mutations and phenotype–genotype correlations in the dystrophic subtypes. JJ Med Genet 2007; 44(3): 181–92.
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Periodontal care in patients with Epidermolysis Bullosa
Atarbashi Moghadam F 1, Atarbashi Moghadam S 2, Haerian Ardakani A3, Ahmadi B 4*
1. Assistant Professor, Department of Periodontology, School of Dentistry, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
2. Assistant Professor, Department of Oral Pathology, School of Dentistry Shahid Beheshti University of Medical Sciences, Tehran, Iran.
3. Associate Professor, Department of Periodontology, Member of Social Determinants of Oral Health Research Center, Dental school, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
4. Undergraduate Dental Student, Dental School, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
Received: 26 May 2013 Accepted: 14 July 2013
Epidermolysis bullosa (EB) is the name given to the group of rare genetically diseases characterized by cutaneous and mucosal blistering which sometimes they leave scars too. In some kinds of epidermolysis bullosa severe blisterings occur after scares which make the oral hygiene practice difficult for them and their parents. Microstomia results from scares make the dental procedures difficult for dentists, too. In this way, this study reviewed the main types of this disease, oral hygiene care and possible periodontal treatment in epidermolysis bullosa.
Key words: Cutaneous and mucosal blistering; Epidermolysis bullosa; Periodontal diseases;
Treatment cares
*Corresponding Author: Tel: 09368792807 Email: [email protected]
CASE REPORT
This Paper Shoud be cited as:
Atarbashi Moghadam F, Atarbashi Moghadam S, Haerian Ardakani A, Ahmadi B. Periodontal care in patients with Epidermolysis Bullosa. Yazd Journal of dental research. 2014,2(1),121-127.
