HNO.90470497.ger.abstr-passage-0
abstract
Die Epidermolysis bullosa acquisita ( EBA ) ist eine seltene , erworbene , bulloese Autoimmundermatose . Sie ist durch die Ablagerung von IgG-Autoantikoerpern gegen das Typ- VII-Prokollagen der Verankerungsfibrille und subepidermale Blasenbildung mit Vernarbung charakterisiert . Die Epidemiologie dieser chronischen Erkrankung und ein charakteristisches klinisches Bild koennen zum gegenwaertigen Zeitpunkt aufgrund nur weniger Berichte im Schrifttum nicht genau eingeschaetzt werden . Bulloese Effloreszenzen treten sowohl am Integument als auch an den Schleimhaeuten auf . Die Entwicklung narbiger Verwachsungen der Schleimhaeute der oberen Atemwege und des Oesophagus ist sehr selten und nur im Einzelfall beschrieben . Wir berichten ueber einen 44jaehrigen Patienten mit massiver Blasenbildung an der Augen- , Nasen- , Mund- , Pharynx- und Larynxschleimhaut und vereinzelten Hautlaesionen mit anschliessender Vernarbung , bei dem die Diagnose einer EBA gestellt wurde . Bei interdisziplinaerer konservativer und operativer Zusammenarbeit war es nach verschiedenen frustranen Therapieansaetzen moeglich , die Entwicklung aktiver Laesionen mit darauffolgenden narbigen Verwachsungen und Stenosen u.a. mittels extrakorporaler Photopherese und Ciclosporin-A einzudaemmen .
HNO.90470497.ger.abstr-s1-r1-t2.1-t1.1
result_of
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s1-r2-t1.1-t2.1
result_of
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s2-r1-t2.1-t1.1
associated_with
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s2-r2-t1.1-t2.1
associated_with
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s5-r1-t1.1-t2.1
developmental_form_of
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s5-r2-t2.1-t1.1
surrounds
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s6-r1-t7.1-t4.1
developmental_form_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s6-r2-t7.1-t2.1
location_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r3-t8.1-t3.1
location_of
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r4-t2.1-t1.1
occurs_in
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s6-r5-t2.1-t3.1
associated_with
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r6-t2.1-t8.1
evaluation_of
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r7-t3.1-t8.1
associated_with
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r8-t8.1-t3.1
associated_with
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r9-t8.1-t2.1
location_of
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r10-t8.1-t2.1
associated_with
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r11-t7.1-t8.1
developmental_form_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r12-t8.1-t7.1
surrounds
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-r13-t4.1-t6.1
adjacent_to
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-r14-t4.1-t8.1
conceptual_part_of
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r15-t3.1-t2.1
associated_with
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r16-t6.1-t8.1
adjacent_to
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r17-t5.1-t6.1
adjacent_to
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-r18-t6.1-t7.1
location_of
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-r19-t5.1-t8.1
conceptual_part_of
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r20-t5.1-t7.1
surrounds
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-r21-t7.1-t5.1
developmental_form_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s6-r22-t6.1-t5.1
adjacent_to
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s6-r23-t4.1-t3.1
location_of
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r24-t3.1-t1.1
occurs_in
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s6-r25-t6.1-t4.1
adjacent_to
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s6-r26-t4.1-t2.1
location_of
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r27-t5.1-t3.1
location_of
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r28-t8.1-t6.1
adjacent_to
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-r29-t6.1-t8.1
conceptual_part_of
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r30-t3.1-t8.1
evaluation_of
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r31-t5.1-t2.1
location_of
HNO.90470497.ger.abstr-s6-t5
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r32-t8.1-t9.1
isa
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-t9
HNO.90470497.ger.abstr-s6-r33-t6.1-t3.1
location_of
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r34-t6.1-t2.1
location_of
HNO.90470497.ger.abstr-s6-t6
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-r35-t7.1-t3.1
location_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s6-r36-t2.1-t8.1
associated_with
HNO.90470497.ger.abstr-s7-t2
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s6-r37-t4.1-t7.1
surrounds
HNO.90470497.ger.abstr-s6-t4
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-r38-t7.1-t8.1
conceptual_part_of
HNO.90470497.ger.abstr-s6-t7
HNO.90470497.ger.abstr-s6-t8
HNO.90470497.ger.abstr-s7-r2-t1.2-t3.1
associated_with
HNO.90470497.ger.abstr-s7-t1
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s7-r4-t3.1-t1.2
associated_with
HNO.90470497.ger.abstr-s7-t3
HNO.90470497.ger.abstr-s7-t1