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Raskauspemfigoidi – Ihotautilääkärin Ja Synnytyslääkärin Yhteinen Haaste

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Raskauspemfigoidi – Ihotautilääkärin Ja Synnytyslääkärin Yhteinen Haaste Laura Huilaja, Kaarin Mäkikallio ja Kaisa Tasanen | HARVINAISET SAIRAUDET Raskauspemfigoidi – ihotautilääkärin ja synnytyslääkärin yhteinen haaste raskauksiin ja trofoblastiperäisiin kasvaimiin Raskauspemfigoidi on harvinainen raskausajan liittyviä tapauksia (Semkova ja Black 2009). autoimmuuni-ihosairaus. Istukan tyypin XVII Aikaisemmin raskauspemfigoidista käytettiin kollageenia vastaan muodostuvat vasta-aineet harhaanjohtavaa nimitystä herpes gestationis. aiheuttavat ihon tyvikalvovaurion ja hankalas- ti kutisevan rakkuloivan ihottuman vartalolle Patogeneesi ja raajoihin. Raskauspemfigoidin diagnoosi var- mistetaan erikoissairaanhoidossa ihokudosnäyt- Raskauspemfigoidin patogeneesi on edel- teestä immunofluoresenssitutkimuksella, ja tau- leen tuntematon. MHC-II-luokan (major din aktiivisuuden arvioinnissa voidaan käyttää histocompatibility complex class II) HL- seerumin pemfigoidiantigeeni BP180:n vasta- antigeenien DR3 ja DR4 tai niiden yhdistel- män on osoitettu olevan selvästi yleisempiä aineen määritystä. Systeeminen kortikosteroidi- raskauspemfigoidiin sairastuneilla naisilla hoito on raskauspemfigoidin hoidon kulmakivi, kuin muussa väestössä (Holmes ym. 1983). vaikka lievien oireiden rauhoittamiseen voivat Istukan ja sikiön kudoksissa on äidille vierai- riittää kortikosteroidivoiteet ja antihistamiini. ta, isältä perittyjä kudosantigeeneja, joihin äi- Synnytyksen jälkeen raskauspemfigoidi rauhoit- din immuuni puolustus ei normaalisti reagoi. tuu yleensä itsestään, mutta uusiutuminen seu- Raskauspemfigoidipotilailla istukan trofobas- raavissa raskauksissa on tavallista. Koska pemfi- tisoluissa sekä vesikalvon (amnion) ja suoni- goidiraskauksiin on liitetty ennenaikaisuuden ja kalvon (korion) stroomasoluissa on havaittu sikiön kasvunhidastuman riski, äitiyspoliklinik- MHC-II-luokan molekyylejä, joita ei tavalli- kaseuranta on suositeltavaa. Äideille kerrotaan sesti esiinny istukassa. Istukan ankkurivillus- myös taudin uusiutumisen mahdollisuudesta ten synsytiotrofoblastikerroksen osittaisen seuraavien raskauksien ja ehkäisyvalmisteiden rikkonaisuuden myötä MHC-II-molekyylien käytön yhteydessä. ajatellaan pääsevän kosketuksiin äidin immuu- nijärjestelmän kanssa, mikä herättää (semi) allogeenisen immuunireaktion tyypin XVII Raskauden aikaansaamat, sikiön kasvun ja kollageenimolekyyliä kohtaan (Kelly ym. kehittymisen mahdollistavat immunologiset, 1989a, b ja 1990). hormonaaliset ja verenkierrolliset muutokset Tyypin XVII kollageeni (pemfigoidianti- näkyvät raskaana olevan äidin ihossa monin geeni 180, BP180) on ihon epidermistä ja der- tavoin. Suurin osa näistä raskausmuutoksista, mistä yhdistävien hemidesmosomien tärkeä kuten melasma (maksaläiskä) ja striat (arpi- rakenneproteiini, joka koostuu lyhyestä solun- juovat), ovat niin yleisiä, että niitä pidetään sisäisestä osasta ja kookkaasta solunulkoisesta normaaleina raskauteen liittyvinä muutoksina. osasta (Powell ym. 2005a). Tätä kollageenia Raskauspemfigoidi sen sijaan on harvinainen on ihon tyvikalvovyöhykkeen lisäksi muun raskaudenaikainen autoimmuuni-ihosairaus. muassa istukassa ja sikiökalvoissa. Istukassa Sen esiintyvyydeksi on arvioitu noin 1:50 000 sitä on havaittavissa sytotrofoblastisoluissa raskautta; lisäksi on kuvattu yksittäisiä rypäle- jo raskauden ensimmäiseltä kolmannekselta 731 Duodecim 2013;129:731–8 HARVINAISET SAIRAUDET alkaen (Huilaja ym. 2008). Raskauspemfigoi- sen yhteydessä. Aaltoilevan taudinkulun on dissa vasta-aineet kohdistuvat pääosin samoi- ajateltu liittyvän progesteroniin, jolla on immu- hin tyypin XVII kollageenin epitooppeihin nosuppressiivisia ominaisuuksia. Hormonin kuin pemfigoidissa (Herrero-Gonzalez ym. pitoisuus suurentuu loppuraskaudessa ja vähe- 2006). Tavallisimmin ne suuntautuvat tämän nee erittäin nopeasti synnytyksen yh tey dessä kollageenin suurimman ei-kollageenisen osan (Jenkins ym. 1999, Semkova ja Black 2009). NC16A:n alueella olevaan epitooppiin, mutta Laajimman (87 potilasta) julkaistun raskaus- lisäksi on todettu vasta-aineita, jotka kohdis- pemfigoidiaineiston mukaan oireet kestävät tuvat tyypin XVII kollageenin solunsisäisiin ja keskimäärin 16 viikkoa ja suurimmalla osalla NC16A:n ulkopuolisiin solunulkoisiin osiin äideistä oireet ovat hävinneet kuuden kuukau- (Di Zenzo ym. 2007). Noin 10 %:lla raskaus- den kuluttua synnytyksestä. Oireiden kesto pemfigoidipotilaista on todettu vasta-aineita synnytyksen jälkeen vaihteli tosin kahden vii- myös ihon tyvikalvon toista rakenneproteiinia kon ja 12 vuoden välillä (Jenkins ym. 1999). BP230:aa (pemfigoidiantigeeni 230) vastaan. Tätä muutosta pidetään kuitenkin sekundaari- Diagnoosi ja erotusdiagnostiikka sena ja kliinisesti merkityksettömänä (Semko- va ja Black 2009). Raskauspemfigoidin tyypil- Raskaus ei suojaa tavallisten ihosairauksien liset iho-oireet aiheutuvat istukassa muodos- esiintymiseltä, mutta se voi vaikuttaa oire- tuneiden vasta-aineiden ja ihon tyypin XVII kuvaan. Koska raskauspemfigoidi on erittäin kollageenin ristiinreagoinnista (Shimanovich harvinainen, iho-oireisen odottajan tapauk- ym. 2002, Semkova ja Black 2009). sessa pyritään ensin sulkemaan pois muut oi- reiden (yleensä kutinan) syyt. Raskauspemfi- Oireet goidin diagnostiikka kuuluu erikoissairaanhoi- toon. Diagnoosi perustuu kliiniseen kuvaan ja Raskauspemfigoidi voi puhjeta missä tahan- oireisen ihoalueen reunamilta otetun ihobiop- sa raskauden vaiheessa tai lapsivuodeaikana, sianäytteen immunofluoresenssitutkimukseen. mutta tavallisemmin oireet alkavat raskauden Siinä todetaan taudille diagnostinen komple- toisen tai kolmannen kolmanneksen aikana. mentti C3:n kertymä tyvikalvossa epidermik- Voimakas vatsan alueen kutina alkaa useim- sen ja dermiksen rajalla (KUVA 2). Noin 25– miten navan ympäristöstä. Kutiseville alueille 50 %:ssa näytteistä havaitaan myös lineaarinen ilmaantuu punoittavia monimuotoisia pa- IgG-positiivisuus, mutta tämä ei ole diagnoo- puloita ja läiskiä ja lopulta muutaman viikon sin edellytys (Shimanovich ym. 2002). Moni- kuluessa rakkuloita. Ne vaihtelevat pienistä muotoista raskausihottumaa sairastavalla po- vesikkeleistä laajempiin paksukattoisiin rak- tilaalla ihon immunofluoresenssitutkimuksen kuloihin, mutta osalla raskauspemfigoidipoti- tulos on negatiivinen. Raskauspemfigoidin laista niitä ei esiinny lainkaan (KUVA 1A). Ai- diagnostiikka ei edellytä tavallisen kudosnäyt- kaisemmin vatsan alueelta alkavaa iho-oireilua teen tutkimista, mutta erotusdiagnostiikassa on pidetty tyypillisenä raskauspemfigoidille, siitä voi joissain tapauksissa olla hyötyä. Ras- mutta kymmenen potilasta käsittäneen yhdys- kauspemfigoidia epäiltäessä suositellaan myös valtalaistutkimuksen mukaan myös raajoista seerumin BP180-vasta-ainepitoisuuden mää- lähtevät iho-oireet ovat yleisiä (Castro ym. ritystä (S-pemfiAb, HUSLAB, Helsinki), sillä 2006). Suomalaisia raskauspemfigoidipotilai- se korreloi taudin vaikeusasteeseen ja helpot- ta vuosilta 2002–2011 (12 potilasta) käsitte- taa hoitovasteen arviointia (Sitaru ym. 2007). levässä tutkimuksessamme kaikilla potilailla Koska BP180-vasta-ainetutkimus on herkkä ja oireet alkoivat vatsalta ja 92 %:lle ilmaantui spesifinen raskauspemfigoidille, sitä on esitet- rakkuloita taudin edetessä (Huilaja ym. 2013). ty jopa sairauden seulontatutkimukseksi (Po- Raskauspemfigoidin oireet lievittyvät yleen- well ym. 2005b). sä muutamia viikkoja ennen synnytystä, mutta Raskauspemfigoidin erotusdiagnostiikassa 732 tauti aktivoituu 75 %:lla uudelleen synnytyk- huomioidaan muut raskauteen ja lapsivuode- L. Huilaja ym. A B KUVA 1. A) Raskauspemfigoidi oireilee tyypillisimmin vatsan iholla. Iho-oireilu voi olla monimuotoista, eikä rakkuloita todeta ainakaan taudin alkuvaiheessa. B) Monimuotoinen raskausihottuma on suurin erotusdiag- nostinen haaste raskauspemfigoidin diagnostiikassa. aikaan liittyvät ihosairaudet. Kliiniset oireet, 19 % potilaista) saavat useimmiten riittävän erotusdiagnostiset kriteerit ja hoitovalinnat avun keskivahvoista tai vahvoista kortikoste- tyypillisimmissä raskausajan ihosairauksissa roidivoiteista, ja kutinan helpottamiseen käy- on esitetty TAULUKOSSA (Al-Fares ym. 2001, tetään lisäksi antihistamiineja, tavallisimmin Kroumpouzos ja Cohen 2003, Ambros-Ru- setiritsiiniä ja hydroksitsiiniä (Jenkins ym. dolph ym. 2006, Rudolph ym. 2006, Beard ja 1999, Shimanovich ym. 2002). Millington 2012). Raskauspemfigoidin suurin Kortikosteroidihoito on vakiintunut hanka- erotusdiagnostinen haaste on monimuotoinen laoireisen raskauspemfigoidin ensisijaiseksi sys- raskausihottuma (polymorphic eruption of teemihoidoksi hyvän hoitovasteen ja turvalli- pregnancy eli PEP), joka tunnettiin aiemmin suusprofiilin vuoksi. Prednisolonista suurin osa nimellä pruritic urticarial papules and plaques inaktivoituu istukan dehydrogenaasin (11-hyd- of pregnancy (PUPPP) (TAULUKKO, KUVA 1B). roxysteroid dehydrogenase-2) vaikutuksesta ennen päätymistä sikiön verenkiertoon. Koska Hoito istukan dehydrogenaasi ei metaboloi fluori- noituja kortikosteroideja (beeta- ja deksame- Raskauspemfigoidin harvinaisuuden vuoksi tasoni), prednisolonia pidetään ensi sijaisena satunnaistettuja tutkimuksia hoidosta ei ole vaihtoehtona (Jackson ym. 2007). Predniso- julkaistu ja hoitosuositukset perustuvat klii- lonin aloitusannos on tavallisesti 20–40 mg/ niseen kokemukseen. Raskauspemfigoidin vrk ja hoitovaste on yleensä hyvä. Mikäli rak- iho-oireet saattavat invalidisoida äitiä hanka- kulamuodostus ei vähene muutamassa vuoro- lastikin, mutta suoranaista terveysvaaraa ras- kaudessa, annosta suurennetaan, kunnes uusia kauspemfigoidista ei hänelle ole. Hoitovalin- rakkuloita ei enää ilmaannu.
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