Porokeratosis - Poroqueratose
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References
Porokeratosis é unha afección cutánea rara caracterizada por problemas de queratinización, que orixinan manchas elevadas en forma de anel ou protuberancias ásperas na pel. A súa característica definitoria ao microscopio é a presenza de lamelas cornoides, unha disposición específica de células na capa superior da pel. Porokeratosis vén en varias formas (disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, linear porokeratosis) . É importante ter en conta que porokeratosis pode converterse en cancro de pel. A mellor forma de diagnosticar porokeratosis é mediante unha biopsia do borde elevado, aínda que actualmente non existe un protocolo de tratamento estándar.
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) é unha enfermidade de queratinización desordenada. É un dos seis tipos de poroqueratose, e normalmente afecta áreas máis grandes en comparación cos outros (linear, Mibelli's, punctate, palmoplantar disseminated, superficial porokeratosis) . O tipo eruptivo de poroqueratose adoita relacionarse co cancro, a inmunidade debilitada ou a inflamación. Os factores de risco inclúen a xenética, a supresión inmunolóxica e a exposición ao sol. O DSAP comeza como protuberancias rosas ou marróns con bordos elevados nas zonas expostas ao sol, que ás veces causan unha lixeira comezón. Os tratamentos varían e poden incluír cremas tópicas, terapia de luz ou medicamentos como 5-fluorouracilo ou retinoides. Estas lesións considéranse precancerosas, cunha probabilidade de 7. 5 - 10 % de converterse en carcinoma de células escamosas ou basocelulares.
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.
Un home de 52 anos, previamente san, entrou cun parche plano en forma de anel na punta do cuarto dedo do pé, que levaba 2 anos sen presentar ningún síntoma. Comezou como un pequeno e duro golpe e foi crecendo ao longo do tempo. A pesar de probar varios tratamentos como crioterapia, cremas, antifúngicos e antibióticos, o parche non mellorou. Ao examinalo detidamente cunha dermocopsia mostrou un centro seco e vermello cun bordo groso e áspero. Un pequeno anaco de pel tomado do bordo do parche mostrou un crecemento celular anormal na capa externa da pel, confirmando un diagnóstico de 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.
A miúdo realízase unha biopsia porque pode parecerse á queratose actínica ou ao carcinoma de células escamosas.