Porokeratosis - Porokeratoos
https://en.wikipedia.org/wiki/Porokeratosis
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References
Porokeratosis 30335323 NIH
Porokeratosis on haruldane nahahaigus, mida iseloomustavad keratiniseerumisprobleemid, mille tulemuseks on annular (ring‑shaped) laigud või karedad punnid nahal. Selle määravaks tunnuseks mikroskoobi all on kornoidlamell, mis on rakkude spetsiifiline paigutus naha ülemises kihis. Porokeratosis esineb erinevates vormides, näiteks levikuline pindmine aktiinsed porokeratoos (disseminated superficial actinic porokeratosis), klassikaline Mibelli porokeratoos (classical porokeratosis of Mibelli), porokeratoos palmaris plantaris et disseminatum (porokeratosis palmaris plantaris et disseminatum) ja lineaarne porokeratoos (linear porokeratosis). Oluline on märkida, et porokeratosis võib potentsiaalselt areneda nahavähiks. Parim viis porokeratosis diagnoosimiseks on biopsia kõrgendatud äärest, kuigi praegu pole standardset raviprotokolli.
Porokeratosis is an uncommon dermatologic disorder. It is a disorder of keratinization that presents with keratotic papules or annular plaques with an elevated border. It has a distinct histologic hallmark of cornoid lamella, which is a column of tightly fitted parakeratotic cells in the upper epidermis. There are multiple clinical variants of porokeratosis, including disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, and linear porokeratosis. Porokeratosis is a precancerous lesion that can undergo malignant transformation. Evaluation of porokeratosis is best with a biopsy of the elevated border. There are no standard guidelines for treatment.
Disseminated Superficial Actinic Porokeratosis 29083728 NIH
Disseminated superficial actinic porokeratosis (DSAP) on ebakorrapärase keratiniseerumise haigus. See on üks kuuest porokeratoosi tüübist ja see mõjutab tavaliselt suuremaid piirkondi võrreldes teistega (linear, Mibelli's, punctate, palmoplantar disseminated, and superficial porokeratosis). Porokeratoosi eruptiivne tüüp on sageli seotud vähi, nõrgenenud immuunsüsteemi või põletikuga. Riskitegurid hõlmavad geneetikat, immuunsüsteemi pärssimist ja päikese käes viibimist. DSAP algab tõusnud servadega roosade või pruunide punnidena päikese käes avatud aladel, põhjustades mõnikord kerget sügelust. Ravimeetodid on erinevad ja võivad hõlmata paikseid kreeme, valgusteraapiat või ravimeid, nagu 5-fluorouracil või retinoidid. Neid kahjustusi peetakse vähieelseteks ja nende tõenäosus on 7,5–10 % muutuda lamerakk- või basaalrakuliseks kartsinoomiks.
Disseminated superficial actinic porokeratosis (DSAP) is a disease of disordered keratinization. Disseminated superficial actinic porokeratosis is one of six variants of porokeratosis. It has more extensive involvement than most other variants. These other variants include linear porokeratosis, porokeratosis of Mibelli, punctate porokeratosis, porokeratosis palmaris et plantaris disseminata, and disseminated superficial porokeratosis. The eruptive form of porokeratosis is associated with malignancy, immunosuppression, and a proinflammatory state. Risk factors for porokeratosis include genetics, immunosuppression, and ultraviolet light. The lesions in disseminated superficial actinic porokeratosis start as pink to brown papules and macules with a raised border in sun-exposed areas that can be asymptomatic or slightly pruritic. There are many options for the treatment of disseminated superficial actinic porokeratosis, including topical diclofenac, photodynamic therapy (PDT), 5-fluorouracil (5-FU), imiquimod, vitamin D analogs, retinoids, and lasers. These lesions are considered precancerous. There is a 7.5 to 10% risk of malignant transformation to squamous cell carcinoma or basal cell carcinoma.
Porokeratosis of Mibelli - Case reports 33150040 NIH
Varem terve 52‑aastane mees tuli sisse lameda rõngakujulise laiguga neljanda varba otsas, mis oli seal olnud 2 aastat ilma mingeid sümptomeid põhjustamata. See algas väikese, kõva muhkena ja kasvas aja jooksul väljapoole. Vaatamata erinevate ravimeetodite, nagu krüoteraapia, topilised kortikosteroidid, seenevastased ravimid ja antibiootikumid, ei muutunud lesioon paremaks. Seda dermokopsiaga tähelepanelikult uurides ilmnes kõrbeline (scaly) atroofiline (atrophic) punane (erythematous) keskosa terava piiri ja hüperkeratoosne (hyperkeratotic) äärega. Lesiooni servast võetud väike nahabiopsia näitas cornoid lamella koos parakeratootiliste rakkudega (parakeratotic cells), mis ulatuvad epidermise invaginatsiooni kaudu, ning granulaarse kihi puudumist (granular layer). See kinnitas Mibelli porokeratoos (Porokeratosis of Mibelli) diagnoosi.
A 52-year-old man with no past medical history presented with an asymptomatic annular atrophic patch on the distal portion of the fourth toe of 2 years’ duration. The lesion began as a small keratotic papule that gradually enlarged centrifugally. He had received multiple treatments including cryotherapy, topical corticosteroids, antifungals, and antibiotics without improvement. Dermoscopic examination revealed a scaly atrophic erythematous central area with a sharply demarcated peripheral hyperkeratotic structure. A skin biopsy of the edge of the lesion revealed a cornoid lamella with a column of parakeratotic cells extending from an invagination of the epidermis with absence of granular layer. The clinicopathologic correlation was consistent with porokeratosis of Mibelli.
Sageli tehakse biopsia, kuna see võib sarnaneda aktiinilise keratoosi või lamekarkkartsinoomiga.