ss - 2009 - philipps-universität marburg · anti-ssb(la) sjögren-syndrom • anti-u 1 rnp...
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Autoimmundermatosen
SS - 2009
Klinik für Dermatologie und Allergologie
Philipps-Universität Marburg
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Kollagen VII
BP180BP230Laminin 5
Dermoepidermale Adhäsion
Desmosom(Desmoglein 1/3)
Epidermis
Dermis
Basalmembran
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Klassifikation blasenbildender Autoimmundermatosen
• Pemphigus-Gruppe• Pemphigoid-Gruppe• Lineare IgA-Dermatosen• Dermatitis herpetiformis Duhring• Epidermolysis bullosa acquisita
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Kollagen VII
BP180BP230Laminin 5
Pemphigus-Formen
Desmosom(Desmoglein 1/3)
Epidermis
Dermis
Basalmembran
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DesmogleinDesmoglein
DesmocollinDesmocollin
PlakoglobinPlakoglobin
CM= Cellmembrane
CM CM
CorePlaque Plaque
KeratinKeratinPlakophilin Plakophilin
Desmoplakin DesmoplakinEnvPerEnv
Per
Env = EnvoplakinPer = Periplakin
Pemphigus - Autoimmunerkrankunggegen desmosomale Adhäsionsmoleküle
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Pemphigus
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Pemphigus-Erkrankungen
Pemphigus vulgarisPemphigus foliaceus
IgA PemphigusParaneoplastischer PemphigusArzneiinduzierter Pemphigus
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Pemphigus vulgaris
Autoantigen(e):
Desmoglein 3
Desmocollin
Plakoglobin
DesmoplakinPlakophilin
EnvPer
Desmoglein 1
Plakoglobin
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Pemphigus vulgaris
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Pemphigus vulgaris
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Pemphigus foliaceus
Autoantigen
Desmoglein 3
Desmocollin
Plakoglobin
DesmoplakinPlakophilin
EnvPer
Desmoglein 1
Plakoglobin
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Pemphigus foliaceus
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Kompensationstheorie beim Pemphigus
Dsg1 Dsg1
Dsg3
Dsg3
Schleimhaut Haut
Mahoney et al, J Clin Invest 1998
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Pemphigus vulgaris
Dsg1
Dsg3
Haut
Dsg1
Dsg3
Mucosa
Dsg3
Dsg3
Dsg1
Dsg1
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Pemphigus foliaceus
Dsg1
Dsg1
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Impetigo contagiosa und Staphylococcal Scalded Skin Syndrom
Enzymatischer Abbau von Desmoglein 1 durch Staphylokokken-Exotoxin
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IgA-Pemphigus
Autoantigene
Desmoglein 3
Plakoglobin
DesmoplakinPlakophilin
EnvPer
Desmoglein 1
Plakoglobin
Desmocollin 1-3
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PD Dr. J. Grabbe, Lübeck
IgA-Pemphigus
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•Schwere Schleimhautbeteiligung
•Polymorphe Exantheme- Blasen- Erosionen- EEM-artiges Bild
•Tumorerkrankung obligat
Anhalt et al 1990; N. Engl. J. Med CLL 18%
Castleman16%
Sarkom 7%
M. Waldenstrom4%
Thymom9%
Lymphom46%
Paraneoplastischer Pemphigus (PNP)
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Paraneoplastischer Pemphigus (PNP)
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Autoantikörperprofil beim paraneoplastischen Pemphigus
Periplakin/EnvoplakinDesmoplakin I,IIHD1/PlektinBP230Desmoglein 1Desmoglein 3170 kD Protein
IC
EC
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B-Zelle
Anti-Dsg1/Dsg3 IgG-Antikörper sind pathogen
Neonataler Pemphigus
Chowdhury, et al., BJD 139:500-503, 1998
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Kollagen VII
BP180BP230Laminin 5
Desmosom(Desmoglein 1/3)
Epidermis
Dermis
Basalmembran
Pemphigoid-Erkrankungen
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Pemphigoid-Erkrankungen
Bullöses PemphigoidPemphigoid gestationis
SchleimhautpemphigoidLineare IgA-Dermatose
Chronisch bullöse Dermatose des Kindesalters
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BP180
BP230
Basalzellen
Pemphigoid – Autoimmunerkrankung gegen hemidesmosomale Adhäsionsmoleküle
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Bullöses Pemphigoid
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Immunpathogenese des Pemphigoid
. CD4+TH1
B-ZelleCD4+TH2
IgG1IgG4
C3
• Autoantikörperbindung an BP180/BP230• Komplementfixierung/-aktivierung• Einwanderung von Granulozyten/Makrophagen• Proteolytische Degradation von Komponenten der BM
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Pemphigoid (Herpes) Gestationis
• 2./3. Trimenon
• initial oft urtikarielle Plaques
• starker Juckreiz
• kreuzreaktive Plazentaantigene?
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Pemphigoid (Herpes) Gestationis
• 5-10% der Neugeborenen ebenso betroffen
• spontane Rückbildung nach Entbindung
• Wiederauftreten in folgenden Schwangerschaften
• Meidung von Kontrazeptiva
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Schleimhautpemphigoid(früher vernarbendes Pemphigoid)
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OkuläresSchleimhautpemphigoid
Stadium I: Chronische Konjunktivitis
Stadium II: Verkürzung der Umschlagfalte von Konjunktiva bulbi auf Bindehaut d. unt. Augenlids
Stadium III: Verwachsung der Lidränder mitdem Augapfel (Symblepharon)
Stadium IV: Verwachsung von oberen undunterem Lidrand, schweres Sicca-Syndrom
Foster et al, Trans Am Ophthalmol Soc 1986
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Kindliches bullöses Pemphigoid
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Lineare IgA-Dermatose
und
Chronisch bullöse Dermatose im Kindesalter
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Lineare IgA-Dermatose des Erwachsenenalters
Klinische Variabilität
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Chronische bullöse Dermatose des Kindesalters
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Dermatitis herpetiformis Duhring
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Dermatitisherpetiformis
Duhring
• oft herpetiformangeordnete Bläschen
• starker Juckreiz
• Assoziation mit gluten-sensitiver Enteropathie
• Autoantikörper gegen epidermale Transglutaminase
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Dermatitisherpetiformis
Duhring
polymorphes klinisches Bild mit Bläschen, Blasen, urtikariellen Plaques und Kratzeffekten
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Dermatitisherpetiformis
Duhring
• Ansammlung eosinophiler und neutrophilerLeukozyten in den dermalen Papillenspitzen
• Granuläre IgA- und Komplementablagerungen
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Kollagen VII
BP180BP230Laminin 5
Epidermolysis bullosa aquisita
Desmosom(Desmoglein 1/3)
Epidermis
Dermis
Basalmembran
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Epidermolysis bullosa acquisita
BP180
BP230
Basalzellen
Kollagen VII
Basalmembran
Antigen unterhalb der Basalmembran:Obligate Vernarbung !
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Diagnostik - Übersicht
Ebene derSpaltbildung
Nachweis gewebegebundenerAutoantikörper
Nachweis zirkulierenderAutoantikörper
Serologische Bestätigungsteste,
Antigen-Differenzierung,
Verlaufsparameter
Klinik/Anamnese!
Diagnose
HistologieDirekte
ImmunfluoreszenzIndirekte
Immunfluoreszenz
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Diagnostik – Histologie
Pemphigus
Pemphigoid/ lineare IgA-DermatoseEpidermolysis bullosa acquisita
Dermatitis herpetiformis
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Diagnostik – Gewebegebundene Antikörper (direkte Immunfluoreszenz)Pemphigus
Pemphigoid/ lineare IgA-DermatoseEpidermolysis bullosa acquisita
IgA, C3
Dermatitis herpetiformis Duhring
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Diagnostik – Serum-Antikörper (indirekte Immunfluoreszenz)
Kollagen VIIBP180BP230
KollagenVII
BP180BP230
1 M NaClBP180, BP230 Laminin-5 Kollagen
VII
DesmoplakineDesmosomale Ag
Affenösophagus/RattenblaseEndomysium
Kochsalz-Spalthaut
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
• systemisch-entzündliche Erkrankungen des Bindegewebes
• aufgrund einer hyperergen Immunreaktion
• mit generalisierter Ausbreitung
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
1. Sklerodermie• zirkumskript (Morphea)• systemische Sklerose2. Lupus erythematodes• kutaner LE• subakut-kutaner LE• systemischer LE3. Dermatomyositis4. Überlappungssyndrome (Mixed connective tissue disease)
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
1. Sklerodermie• zirkumskript (Morphea)• systemische Sklerose2. Lupus erythematodes• kutaner LE• subakut-kutaner LE• systemischer LE3. Dermatomyositis4. Überlappungssyndrome (Mixed connective tissue disease)
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Zirkumskripte Sklerodermie (Morphea)
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Zirkumskripte Sklerodermie (Morphea)
• lokalisierte überschiessende Kollagensynthese
3 Stadien:• Stadium erythematosum• Stadium indurativum• Stadium atrophicans
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Formen der zirkumskripten Sklerodermie• Plaqueförmig
• Lineäre (streifiger) Morphea(Maximalvariante mit Atrophievon Muskulatur und Knochen: „Coup de sabre“)
• Eosinophile Fasziitis(Shulman-Syndrom)
• Generalisierte Morphea(pansklerotisch)
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Zirkumskripte Sklerodermie - Therapie
• topische Glukokortikoide (extern, intrakutan)• Penicillin G i.v.• ggf. Immunsuppressiva• Borreliose ausschliessen!• symptomatisch (Physiotherapie, Massage)• UVA1-, PUVA-Bestrahlung
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Systemische Sklerose
Multilokale Überproduktion von Kollagen und Bindegewebssubstanzen -> Leitsymptom: Fibrose!
• Sklerodermie (peripher, zentral)• Nephrosklerose,
nephrogener Hypertonus• Ösophagusmotilitätssstörung• Lungenfibrose• Myokard-, Perikardfibrose
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Systemische Sklerose - Akrosklerose/Sklerodaktylie
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Systemische Sklerose - Faziale Sklerose
• Mikrostomie• Amimie• verkürztes Frenulum
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Systemische Sklerose
• anfallsartige, schmerzhafte Gefäss-Spasmender Fingerarterien
• häufig Initialsymptom
• Triggerung durch physikalische (Kälte, Vibration) oder psychische Faktoren (Stress)
Mikroangiopathie -> Raynaud-Phänomen
WeissBlauRot
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Systemische Sklerose
Mikroangiopathie -> trophischeSchäden
• Onychodystrophie und Onycholyse• Fingerkuppenulzera• Endgliednekrosen
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Systemische Sklerose - Calcinosen
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Systemische Sklerose
Polyklonale Aktivierung des Immunsystems -> Autoantikörper
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Klinische Einteilung
Limitierte Form:• Sklerose von Akren und Gesicht mit möglicher zentrifugaler
Ausbreitung• Sonderform CREST-Syndrom (Calcinosis cutis, Raynaud-Phänomen,
Ösophagusmotilität↓, Sklerodaktylie, Teleangiektasien)• Autoantikörper: anti-Centromeren-Ak
Diffuse Form:• Sklerose der gesamten Haut; Multiorganbefall• Autoantikörper: anti-Scl-70-Ak
Systemische Sklerose
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Systemische Sklerose - Therapie
• Fibrose: Penicillin G, PUVA-Bestrahlung, Massage
• Mikrozirkulation: Ca-Antagonisten, ACE-Hemmer, Calcitonin, Prostaglandine, Rheologica
• Immunsuppressiv/antientzündlich: Glukokortikoide, andere Immun-
suppressiva (insbes. Cyclophosphamid)
• Symptomatisch: Physiotherapie, Wärme, Kälteschutz, Antacida
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
1. Sklerodermie• zirkumskript (Morphea)• systemische Sklerose2. Lupus erythematodes• kutaner LE• subakut-kutaner LE• systemischer LE3. Dermatomyositis4. Überlappungssyndrome (Mixed connective tissue disease)
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Chronisch kutaner Lupus erythematodes (CDLE)
• Erythem, festhaftende Schuppung, Atrophie
• schubartiger Verlauf
• vorwiegend lichtexponierte Areale
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Subakut kutaner Lupus erythematodes (SCLE)
• Flüchtige Exantheme
• Photosensitivität
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Systemischer Lupus eryxthematodes (SLE)
• Multiorganerkrankung
• Haut:persistierende Erythememakulourtikarielle ExanthemePhotosensitivität
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Kutane Symptome beim SLE
Livedovaskulopathie periungual schuppende Erytheme
Enanthem,Erosionen
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Diagnosekriterien des sytemischen Lupus erythematodes(ACR-Kriterien 1982, revidiert 1997)
SchmetterlingserythemDiskoides ExanthemPhotosensibilitätOrale/nasale Schleimhaut-UlzeraArthritisSerosititsNephropathieZNS-BeteiligungHämatologische VeränderungenImmunologischeVeränderungenAntinukleäre Antikörper (ANA)
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Systemischer Lupus erythematodesÄtiologie
• Genetische Faktoren(familiäre Häufung, vorwiegend Frauen)
• Hormone(Östrogene verschlechternd, Androgene mildernd)
• Exogene Faktoren(UV-Licht, Medikamente)
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Systemischer Lupus erythematodes - Pathogenese
Vessels
• polyklonale B-Zellaktivierungmit Produktion von Auto-Ak
• Bildung von Immun-komplexen mit Aktivierung des Komplementsystems
Lupusband
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Autoantikörper beiLupus Erythematodes
• Antinukleär (ANA) unspezifisch• Anti-dsDNS SLE (systemisch)• Anti-SM SLE (Nephritis)• Anti-SSA (Ro), CDLE, SCLE,
anti-SSB(La) Sjögren-Syndrom• Anti-U1RNP Overlap-Syndrome• Anti-H2A,anti-H2B arzneiinduzierter LE
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Neonataler Lupus erythematodes
anti-Ro (SSA) bzw. anti-La (SSB)-Antikörper sind pathogen
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Neonataler Lupus Erythematodes
AV-Block, Myokarditisund Perimyokarditis
Hepatitis oder Thrombopenie
HV wie beim subakut kutanen LE
Symptome:
Kalretikulin
Ca2+ -Kanäle im Myozyt
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Therapieoptionen beim Lupus erythematodes
• Kutan (umschrieben): LichtschutzGlukokortikoide
• Kutan (disseminiert): HydroxychloroquinGlukokortikoideRetinoideDADPS, (Thalidomid)
• Systemisch: GlukokortikoideImmunsuppressivaAlkylantien (Fauci-Schema)Sonstige (Immunadsorption, IVIG, anti-CD20-Antikörper)
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
1. Sklerodermie• zirkumskript (Morphea)• systemische Sklerose2. Lupus erythematodes• kutaner LE• subakut-kutaner LE• systemischer LE3. Dermatomyositis4. Überlappungssyndrome (Mixed connective tissue disease)
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Dermatomyositis
Myositis mit charakteristischen Hautveränderungen
Typ I Adulte Polymyositis (PM)Typ II Adulte Dermatomyositis (DM)Typ III Paraneoplastische PM/DMTyp IV Juveline Dermatoyositis (DM)Typ V Assoziierte PM/DMTyp VI Amyopathische Dermatomyositis
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Dermatomyositis - Hautveränderungen
Heliotropum aborescens
Heliotroperythem
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Dermatomyositis - Hautveränderungen
Gottron‘sche Papeln
Keining‘sches Zeichen
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Dermatomyositis - HautveränderungenCalcinosis cutis
Poikilodermie
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Dermatomyositis - Diagnostik
• klinische Untersuchung• Kreatinkinase, BSG• Elektromyogramm• Muskelbiopsie• Autoantikörper
(ANA, Anti-Jo1-Ak, Anti-Mi2-Ak)
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Dermatomyositis - Therapie
- Immunsuppressiva(Kortikosteroide, Azathioprin, Methotrexat, etc.)
- Immunglobuline i.v.
- maßvolle physikalische Therapie
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Kollagen-Gefässerkrankungen(Kollagenosen/ Collagen Vascular Disorders)
1. Sklerodermie• zirkumskript (Morphea)• systemische Sklerose2. Lupus erythematodes• kutaner LE• subakut-kutaner LE• systemischer LE3. Dermatomyositis4. Überlappungssyndrome (Mixed connective tissue disease)
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Mixed connective tissue disease
• klinische Aspekte von Sklerodermie, SLE und DM
• meist entzündliche, sklerodermieartige Hautveränderungen
• Arthralgien, Myalgien
• “Umwandlung” in reineSklerodermie oder SLE möglich
Sklerodaktylie – „Mechanic’s hands“