Porokeratosis - Porokeratose
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De harde, utstikkende kantene er karakteristiske.
relevance score : -100.0%
ReferencesPorokeratosis er en sjelden hudtilstand preget av keratiniseringsforstyrrelser, som gir hevede, ringformede flekker eller grove støt på huden. Dens karakteristiske mikroskopiske funn er tilstedeværelsen av cornoid lamell, et spesifikt arrangement av celler i hudens øverste lag. Porokeratosis forekommer i ulike former (disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, linear porokeratosis). Det er viktig å merke seg at porokeratosis kan utvikle seg til hudkreft. Den mest pålitelige diagnostiske metoden er en biopsi av den opphøyde kanten, selv om det foreløpig ikke finnes noen standard behandlingsprotokoll.
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.
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 (DSAP) er en sykdom med forstyrret keratinisering. Det er en av seks typer porokeratose, og den påvirker vanligvis større områder enn de andre (linear, Mibelli's, punctate, palmoplantar disseminated, superficial porokeratosis). Den eruptive typen av porokeratose er ofte knyttet til kreft, svekket immunforsvar eller betennelse. Risikofaktorer inkluderer genetikk, immunundertrykkelse og soleksponering. DSAP starter som rosa eller brune nupper med hevede kanter i soleksponerte områder, og kan noen ganger forårsake lett kløe. Behandlinger varierer og kan omfatte aktuelle kremer, lysterapi eller medisiner som 5‑fluorouracil eller retinoider. Disse lesjonene anses som precancerøse, med en 7,5–10 % sjanse for å utvikle plateepitel‑ eller basalcellekarsinom.
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.
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.
En 52 år gammel mann, tidligere frisk, oppsøkte oss med et flatt, ringformet plakk på enden av fjerde tå, som hadde vært til stede i 2 år uten symptomer. Det startet som en liten, hard hevelse og vokste gradvis over tid. Til tross for ulike behandlinger, inkludert kryoterapi, kremer, soppdrepende midler og antibiotika, ble ikke plakket bedre. Ved nøye undersøkelse med dermoskopi viste den et tørt, rødt senter med en tykk, grov kant. Et lite hudprøve tatt fra kanten av plakket viste unormal cellevekst i det ytre hudlaget, noe som bekreftet diagnosen porokeratosis of Mibelli.
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.
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.
Ofte tas en biopsi fordi lesjonene kan ligne aktinisk keratose eller plateepitelkarsinom.