Porokeratosis - Porokeratose
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ReferencesPorokeratosis er en sjælden hudlidelse, som er karakteriseret ved keratiniseringsproblemer, hvilket resulterer i hævede, ringformede pletter eller ru knopper på huden. Dens definerende mikroskopiske træk er tilstedeværelsen af cornoid lameller, et specifikt arrangement af celler i hudens øverste lag. Porokeratosis forekommer i forskellige former, såsom disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum og linear porokeratosis. Det er vigtigt at bemærke, at porokeratosis potentielt kan udvikle sig til hudkræft. Den mest pålidelige måde at diagnosticere porokeratosis på er ved en biopsi af den hævede kant, selvom der i øjeblikket ikke findes en standardbehandlingsprotokol.
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 sygdom med forstyrret keratinisering. Det er en af seks typer af porokeratose, og den påvirker typisk større områder sammenlignet med de andre (linear, Mibelli's, punctate, palmoplantar disseminated, and superficial porokeratosis). Den eruptive type porokeratose er ofte forbundet med kræft, svækket immunitet eller betændelse. Risikofaktorer involverer genetik, immunundertrykkelse og soleksponering. DSAP begynder som lyserøde eller brune knopper med hævede kanter i soleksponerede områder, hvilket nogle gange giver let kløe. Behandlingerne varierer og kan omfatte topiske cremer, lysterapi eller medicin som 5-fluorouracil eller retinoider. Disse læsioner betragtes som præcancerøse med en 7,5–10 % chance for at udvikle sig til pladecelle- eller basalcellecarcinom.
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‑årig mand, tidligere rask, kom ind med et fladt, ringformet plak på enden af sin fjerde tå, som havde været der i 2 år uden at give symptomer. Det startede som et lille, hårdt bump og voksede udad med tiden. På trods af, at han havde prøvet forskellige behandlinger såsom kryoterapi, cremer, svampedræbende midler og antibiotika, blev plaketten ikke bedre. Ved nøje undersøgelse med dermokopi viste den et tørt, rødt centrum med en tyk, ru kant. Et lille hudstykke fra kanten af plaketten afslørede unormal cellevækst i det ydre hudlag, hvilket bekræftede 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 udføres en biopsi, fordi den kan ligne aktinisk keratose eller planocellulært karcinom.