pachidermoperiostosis

pachidermoperiostosis (pidipi) anedi emukalu mariyu charmam rendintinee prabhaavitam chese arudaina janyu rugmata. itara paerlu praadhamika hypertrophic austio aarthropathi ledhaa tourain-solent-goal syndrome. idhi pradhanamgaa pachiderma (charmam gattipadatam), periostosis (adhika emuka nirmaanam) mariyu vaeli kalayika (goru mariyu goru mancham madhya saadhaarana koonaanni kolpovadamtho kanajaalam vaapu) kaligi untundi.
eee vyaadhi mahilala kante purushulanu ekkuvagaa prabhaavitam chestundi. praarambhamaina tarvaata, vyaadhi sumaru 5-20 samvatsaraala tarvaata sthireekarinchabadutundi. pidipi rogula jeevitam teevramgaa debbatintundi. prastutam, rogalakshana chikitsalu ensides mariyu steroids ledhaa shastrachikitsa vidhanalu.
vidhanalu, pidipini modatisaarigaa friedreach 'mottam asthipanjaram yokka emuka adhika perugudala' gaa abhivarninchaaru. tourain, solent mariyu goal goal pidipini emuka vyaadhi hypertrophic osteoarthropathy yokka praadhamika roopamgaa abhivarninchaaru mariyu daani moodu telisina roopaalanu vaeru chesaaru.
lakshanaalu.
pdplo aneka kanipinche sankethaalu unnaayi. atyanta mukhyamaina vaidya lakshanaalu pachidermia (charmam gattipadatam mariyu mudatalu padatam), mukham mariyu charmam yokka bochu, periostosis (periorticular kanajaalam vaapu mariyu podavaina emukala yokka shaagi periostial kotta emuka nirmaanam) mariyu digital clubbing (velikonalu peragadam). itara lakshanaalalo adhika chemata pattadam, arthralgia mariyu jeernasayaantara asaadhaaranatalu unnaayi. anni lakshanaala yokka avalokanam pattika pattika andinchabadindi.
pattika 2. sankethaala avalokanam 
kaaranam.
sthaanikamgaa panichese madhyavarti prostaglandin e2 (pge2) pdp yokka vyaadhikaarakamlo paatra poeshistumdi. pidipi rogulalo, adhika sthaayi rogulalo mariyu taggina sthaayi pge-emm pge-emm yokka metabolite) gamaninchabaddaayi.
pge2 osteoblastlu mariyu osteoclastla (varusaga emuka kanajaalaanni nirminchi vichchinnam cheyadam) charyanu anukarinchagaladu. anduke accrustiolysis mariyu periostial emuka nirmaanaanni pge2 charya dwara vivarinchavacchu. inca, pge2 vasodilatory prabhavalanu kaligi untundi, idhi digital clubbinglo deerghakaalika sthaanika vasodilationku anugunamgaa untundi.
pidipi rogulalo rogulalo yokka perigina sthaayilu hetch. pii. zii. dii janyuvu yokka utparivartanaalato mudipadi untaayi. eee rogulu digital clubbing mariyu periostosis vanti saadhaarana pidipi lakshanaalanu chuupimchaaru. hetch. pii. zii. dii janyuvu chromosome chromosome paie mapp cheyabadutundi mariyu enjaim hetch. pii. zii. dii (15-hydroxyprostaglande dehydrogenase) nuu encode chestundi. eee enjaim pge2 mariyu sambandhita icosanoids ksheenatalo modati dasanu utprerakam chestundi. ippativaraku, enimidi ververu utparivartanalu pidipi rogulalo panicheyani hetch. pii. zii. dii enjaimku daariteesaayi. eee utparivartanala kaaranamgaa, pge2 uparitalaanni uparitalaanni bandhinchadam antaraayam kaligistundi. dheeni phalitamgaa, pge2 nii pge-m down loki badilee cheyalemu mariyu adhika saandratala vadda untundi.
itara madhyavartula paatra.
adhika pge2 sthaayilatho paatu, hypertrophic osteoarthropathy unna rogulalo chesina adhyayanalu vaan willebrand karakam mariyu vascular endothelial groth phyaaktar (wezf) vanti aneka itara madhyavartula plasma sthaayilu kooda periginatlu choopinchaayi. eee padaarthaalu pidipi purogati mariyu vistaranalo kooda paatra pooshinchavachchu. hetch. pii. zii. dii utparivartanalaku viruddhamgaa, eee kaarakaalaku anumaanaaspada utparivartanalu inca nivedinchabadaledu.
vaan willebrand karakam anedi platelet mariyu endothelial kriyaaseelataku oka marker. endothelial kanaalu mariyu plateletla kriyaaseelata pidipi yokka vyaadhikaarakamlo mukhyamaina paatra pooshistundani idhi suuchistumdi. wezf angiogenesis (kotta rakta naalaala perugudala) mariyu osteoblastla bhedaanni prothsahistundi, idhi pidipi rogulalo clubbing mariyu adhika fibroblast erpadataanni vivaristundi.
pidipi rogulalo perigina saandratalaloo kanipinche itara madhyavartulalo osteocalacin, endotheline-1, bii-thromboglobulin, platelet-utpanna perugudala karakam (pdf) mariyu epidermal perugudala karakam (egf) unnaayi. pdplo eee madhyavartula paatra emito inca vivarinchaledu.
janyusaastram.
eee paristhithitho rendu janyuvulu mudipadi unnaih chromosome 4 (4q34.1) loe hydroxyprostaglande dehydrogenase 15-(enedi) (hechpijidi) mariyu chromosome 3 (3q22.1-q22.2) loe draavakam vaahaka sendriya ayan ravana kutumba sabhyudu 2a1 2a1. rendu janyuvula kaapeelu utparivartana chendinatlayithe (autosomal recessive inheritance) eee syndrome sambhavistundi.
rooga nirdhaarana.
podavaina emukala pachidermia, vaeli kalayika mariyu periostosis unnappudu pidipini nirdhaarinchadaaniki sulabhamaina maargam. podavaina emukala radiographla dwara periostium kinda kotta emuka erpadataanni gurtinchavacchu. pidipini nirdhaarinchadaaniki, tarachugaa itara vyaadhulanu minahaayinchaali. udaaharanaku, dviteeya hypertrophic austio aarthropatini minahaayinchadaaniki, hridayanaala, pulmonary, hepatic, pegu mariyu madhyastha vyaadhula sankethaalu tappanisarigaa undakudadu. mri mariyu ultrasound kooda lakshanaala phalitaalanu kaligi untaayi.
charma biopsy anedi pidipini nirdhaarinchadaaniki maroka maargam. ayithe, idhi chaalaa nirdishtamaina paddhati kaadu, endukante itara vyaadhulu pidipitho adhe charma maarpulanu panchukuntaayi, mixedema mariyu hypothyroidism vantivi. eee itara vyaadhulanu minahaayinchadaaniki, harmonela adhyayanalu jarugutaayi. udaaharanaku, thyroid akropachi mariyu akromegaaleeni minahaayinchadaaniki thyrotropine mariyu groth harmon sthaayilanu pariseelinchaali. ayithe, charma biopsy anedi charma lakshanaalu laeni rogulalo pidipini nirdhaarinchadaaniki sahaayapadutundi.
clubbing gamaninchinappudu, vella yokka dhoorapu falangla accrustiolysis undaa anie tanikhi cheyadam sahaayapadutundi. idhi pidipini nirdhaarinchadaaniki upayogapadutundi, endukante clubbing mariyu accrustiolysis kalayika pidipi mariyu chenee syndromelo matrame kanipistundi.
biomarkerlu mariyu utparivartana vislaeshanha.
perigina pge2 sthaayilu mandulu paraspara sambandham kaligi unnanduna, moothra pge2 eee vyaadhiki upayogakaramaina biomarker kaavacchu. adanamgaa, hetch. pii. zii. dii. utparivartana vislaeshanhalu saapekshamgaa choukainavi mariyu saralamainavi mariyu vivarinchaleni clubbing unna rogulalo ledhaa pii. dii. pii-lanty lakshanaalanu pradarsinche pillalalo prarambha parisoedhanaloo upayogakaramgaa niroopinchabadavacchu. prarambha saanukuula phalitaalu pathologyni gurtinchadamlo khareedaina mariyu deerghakaalika pareekshalanu nirodhinchagalavu.
pidipi vyaadhi karyakalapalanu anusarinchadaaniki, tapp, baap, bgp, karbadi terminal propeptide rakam ii procologen ledhaa ntx vanti emuka nirmaana gurtulu mukhyamaina paatra poeshistaayi. pariganinchagala itara biomarkerlu ill-6 mariyu nf-kebi ligand (rankl) yokka grahaka kriyaaseelakam, ivi kontamandi rogulalo emuka punarviniyogamto mudipadi untaayi. ayithe, vyaadhi paryaveekshanha yokka eee upayogaanni nirdhaarinchadaaniki marinta parisoedhana avasaram.
oopiritittula cancer mariyu vaeli kalayika unna rogulalo kooda pge2 penchavacchu. idhi prostaglandins yokka gvakriyalo palgone enjaim ayina cyclooxygenase-2 yokka perigina sthaayilaku sambandhinchinadi kaavacchu. systic fibrosislo idhe vidhamaina anubandham gurtinchabadindi.
vargeekarana.
rendu rakaala hypertrophic austio aarthropathi loe pidipi okati. idhi mottam hypertrophic austio aarthropathi kesulalo sumaru 5 saatam praatinidhyam vahistundi. rendava roopam secondary hypertrophic osteoarthropathy (show). shoku saadhaaranamgaa antarleena vyaadhi untundi (cardiopulmonary vyaadhulu, praanaantakaalu ledhaa paranoplastic syndrome vantivi). sholaga kakunda, pidipiki antarleena vyaadhi ledhaa praanaantakam ledhu.
pidipini moodu vargaluga vibhajinchavachhuh
eee bhedaatmaka rogalakshanaalaku kaaranam ippatikee teliyadu.
chikitsa.
niyantrita dayta lekapovadam valla pidipiki samarthavanthamaina chikitsa prastutam teliyadu mariyu idhi ekkuvagaa cases nivedikalapai aadharapadi untundi. hechpijidi enjaim pidipi yokka vyaadhikaarakamlo palgone avakaasam unnappatikee, lopabhuishta enjaimnu parishkarinchadam kashtam kabatti eee utparivartanaku vyatiraekamgaa vyuuhaalu inca nivedinchabadaledu. janyu chikitsa dheeniki parishkaram kaavacchu, ayithe idhi inca saahityamlo nivedinchabadaledu.
vaapu mariyu noppini tagginchadaaniki sampradaaya pidipi aushadha chikitsalo ensides mariyu carticosteroids untaayi. pidipi rogulu upayoginche itara mandulu emuka nirmaanam ledhaa charma aavirbhavaalanu lakshyamgaa chesukuntaayi. soundarya roopaanni meruguparachadaaniki shastrachikitsa samrakshananu upayogistaaru.
vaapu mariyu noppi aushadha chikitsa.
steroid kaani shotha nirodhaka mandulu (ensides) mariyu carticosteroids pidipi chikitsalo ekkuvagaa upayoginchabadataayi. eee mandulu saiklo-oxygenase charyanu nirodhistaayi, tadwara prostaglandin samshlaeshanhanu nirodhistaayi. peristial emuka nirmaanam mariyu accrustiolysislo pge2 palgone avakaasam unnanduna, eee mandulu mandulu sambandham unna paalii artharaitisnu tagginchagalavu. adanamgaa, ensides mariyu carticosteroids inflammatory madhyavartula erpaatunu taggistaayi, vaapu mariyu noppini taggistaayi. saadhyamayye gastropathy vishayamlo, cox-2 selective enside atorixosibcu praadhaanyata istaaru.
infliximab pdplo noppi mariyu artharaitisnu taggistundi. idhi tnf-α (tumour necrosis phyaaktar-alfa) yokka jeeva charyanu nirodhinche monoclonal antibody. tnf-α anedi pdplo adhika sthaayilo kanipinche inflammatory cytokine mariyu idhi itara inflammatory madhyavartula utpattilo paalgontundi, idhi rankle yokka vyakteekarananu penchutundi. rankl emukala punarviniyogaanni penchutundani bhaavistunnaaru.
emuka nirmaanam mariyu noppi aushadha chikitsa.
pomidronate ledhaa risedronate vanti bisphosphonetlatho chikitsa cheyadam dwara rheumatologic lakshanaalanu meruguparachavacchu. bisphosphonatelu osteoclastic emuka punarviniyogaanni nirodhistaayi, anduvalla emuka punarnirmananni taggistaayi mariyu baadhaakaramaina paalii artharaitisnu taggistaayi.
pratyeka sandarbhaallo, tamoxifen pidipi chikitsalo, mukhyamgaa emuka mariyu keella noppiki prabhaavavantamgaa untundi. pidipi rogulalo, steroidla kosam adhika sthaayi nuclear grahakaalu kanugonabaddayi, idhi eastrogen grahaka virodhi ayina tamoxifen nuu upayoginchadaaniki kaaranam. tamoxifen mariyu daani aneka gvakriyalu kanajaala lakshyalapai eastrogen graahakaalato poteegaa bandhistaayi, idhi DNA samshlaeshanhanu tagginche anhu samudaayaanni utpatti chestundi. kanaalu g0 mariyu g1 dasallo perukupotayi. inn vitro adhyayanalu tamoxifen emukapai eastrogen agonistga panichestundani mariyu osteoclastla (emuka kanajaalaaniki antaraayam kaliginchadam) yokka punarviniyoga karyakalapalanu nirodhistundani choopinchaayi.
charma aavirbhavaalu aushadha chikitsa.
charmapu aavirbhavaalanu meruguparachadaaniki retinoidlanu upayogistaaru. retinoids retinoid nuclear grahakaalapai panicheestaayi mariyu tadwara transcription niyantristaayi. udaaharanaku, motimalaku chikitsa cheyadaaniki atyanta prabhaavavantamaina aushadhamaina isotritinoin, maanava sabasius grandhulalo apoptosisnu prerepinchadam dwara soundarya lakshanaalanu meruguparustundi. dheeni phalitamgaa, bandhana kanajaalam perugudala mariyu seabasius grandhula hyperplasia nirodhinchabadutundi. retinoids fibroblastlalo procologen mrnnu kooda taggistaayi, idhi pachidermaanu meruguparustundi.
retinoids maadirigaane, colchisins kooda charmapu aavirbhavaalanu meruguparustaayi. idhi daani podavunu nivaarinchadaaniki suukshma naalikala chivaralanu bandhinchagaladu. suukshma naalikalu kana vibhajana, sanketha badilee mariyu janyu vyakteekarana niyantranalo palgontayi kabatti, kolshisin kana vibhajana mariyu shotha prakriyalanu nirodhinchagaladu (udhaa.zii. neutrophils mariyu leukocytes yokka charya). kolshisin leukocytes yokka chemotactic charyanu nirodhistundani soochinchabadindi, idhi pachidermia taggimpuku daariteestundi.
botulinum taxin rakam eah (bii. tii. ex-eah) vadakam rogula merugaina leonin mukhalanu meruguparichindi. btx-a anedi neuromuscular junction vadda panichese acetylcholine vidudalanu nirodhistundi. antekakunda, idhi chemata grandhulaku colinergic prasaranni nirodhistundi, anduvalla chemata sraavam nirodhistundi. ayithe, leonin mukhalanu meruguparachadaaniki khachitamaina vidhanam teliyadu mariyu marinta daryaaptu cheyalsina avasaram undhi.
shastra chikitsa.
aushadha chikitsalatho paatu, mukha roopaanni meruguparachadaaniki aneka shastrachikitsa paddhatulu unnaayi. vaatilo okati facelift, dheenini saanketikamgaa facial rhytidectomy anie pilustaaru. eee paddhati marinta yavvana roopaanni ivvadaaniki upayoginche oka rakamaina soundarya shastrachikitsa vidhanam. indulo mukhampai unna adanapu charmanni tolaginchadam mariyu mukham mariyu medapai charmanni biginchadam untaayi. rendava empika plaastik surgery. idhi kanti drooping kosam kooda upayoginchabadutundi.
rooga niruupana.
prarambha vayassu tarachugaa yuktavayassulo untundi. vivarinchina kesulalo, 80 saatam mandhi baadhita vyaktulu 18 samvatsaraala vayassulopu eee vyaadhitho baadhapadutunnaaru. ayithe, latos-beelenska mariyu itarulu. eee saatam takkuvagaa undaalani paerkonnaadu, endukante austio aarthropathi yokka maroka roopam-family idiopathic austio aarthropathi (fio)-eee vislaeshanhaloo parigananaloki teesukobadindi.
pidipi saadhaaranamgaa 5 nundi 20 samvatsaraala varaku sthiramgaa unde varaku konasaagutundi. rogulu parimita kadalika, naraala vyakteekaranalu mariyu leonin mukhaalato sahaa aneka kriyaatmaka mariyu soundarya samasyalanu edurkontunnappatiki, ayurdayam saadhaaranam kaavacchu.
epidemiology.
praabalyam.
pidipi anedi arudaina janyu vyaadhi. kaneesam 204 pidipi kesulu namodayyayi. pidipi yokka khachitamaina sambhavam mariyu praabalyam ippatikee teliyadu. 0.16% praabalyam jaajiku mariyu jaajiku suuchimchimdi.
pampinhee.
pidipi anedi mahilala kante purushulalo ekkuvagaa sambhavistundi (nishpatti 7:1). antekakunda, purushulu marinta teevramaina lakshanaalatoo baadhapadutunnaaru (pattika 1 chudandi). african american prajalu ekkuvagaa prabhaavitamavutunnaaru.
pattika 1.201 mandhi baadhita purushulu mariyu mahilalalo (167 mandhi purushulu mariyu 34 mandhi mahilalu) vividha rakaala pidipi pampinhee.
vaarasatvam.
25-38% kesulalo, rogulaku pidipi yokka kutumba charitra untundi. asampoorna roopam mariyu puurti roopam vividha margallo vaarasatvamgaa labhistaayani suchinchabadindihi autosomal dominant inheritance (dominant yugmavikalpamtho kuudina) ledhaa autosomal recessive inheritance (recessive yugmavikalpamtho kuudina).
chochukupovadam mariyu chararashi vyakteekaranhatho kuudina vaarasatvam yokka autosomal aadhipatya namuunaa asampoorna roopamtho sambandham unna sagam kutumbaalalo nirdhaarinchabadindi. aneka kutumbaalalo, keellu, emuka mariyu charma lakshanaalatoo kuudina marinta teevramaina lakshanaalatoo puurti roopamtho anubandhinchabadina autosomal tirogamana namuunaa vaarasatvam yokka telisinadi. pdplo purusha-stree nishpatti purushula vaipu vakramgaa untundi.
eee paristhithitho rendu janyuvulu mudipadi unnaih hydroxyprostaglande dehydrogenase 15-(enedi) (hechpijidi) mariyu draavakam vaahaka sendriya ayan ravana kutumbam, sabhyudu sabhyudu ravana samstha samstha. antarleena pathophysiology prostaglandin ii2 yokka asaadhaaranatagaa kanipistundi, ayithe vivaralu inca spashtam cheyabadaledu.
samajam.
6 roogi samsthalu pidipi rogulaku sahayanni andistaayi. vaatilo 4 airopalo (finland, france, grease mariyu polland) unnaayi. migilina rendu australia mariyu moraco (association marocain dess genodermatoses) loe unnaayi.

