Porokeratosis - Poroceratose
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References
Porokeratosis é uma doença rara da pele caracterizada por problemas de queratinização, resultando em manchas salientes em forma de anel ou inchaços ásperos na pele. Sua característica definidora ao microscópio é a presença da lamela cornóide, um arranjo específico de células na camada superior da pele. Porokeratosis vem em vários formatos, como disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, and linear porokeratosis. É importante observar que porokeratosis pode potencialmente evoluir para câncer de pele. A melhor maneira de diagnosticar porokeratosis é através de uma biópsia da borda elevada, embora atualmente não exista um protocolo de tratamento padrão.
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 uma doença de queratinização desordenada. É um dos seis tipos de poroceratose e geralmente afeta áreas maiores em comparação com os outros (linear, Mibelli's, punctate, palmoplantar disseminated, and superficial porokeratosis) . O tipo eruptivo de poroceratose geralmente está associado ao câncer, imunidade enfraquecida ou inflamação. Os fatores de risco envolvem genética, supressão imunológica e exposição solar. O DSAP começa como inchaços rosados ou marrons com bordas elevadas em áreas expostas ao sol, às vezes causando leve coceira. Os tratamentos variam e podem incluir cremes tópicos, fototerapia ou medicamentos como 5-fluorouracil ou retinóides. Essas lesões são consideradas pré-cancerosas, com 7. 5 - 10 % de chance de se transformarem em carcinoma espinocelular ou basocelular.
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.
Um homem de 52 anos, previamente saudável, apresentou uma placa plana e em forma de anel na ponta do quarto dedo do pé, que estava ali há 2 anos sem causar nenhum sintoma. Começou como uma protuberância pequena e dura e foi crescendo com o tempo. Apesar de tentar vários tratamentos como crioterapia, cremes, antifúngicos e antibióticos, o adesivo não melhorou. O exame minucioso com uma dermatocopsia mostrou um centro seco e vermelho com uma borda espessa e áspera. Um pequeno pedaço de pele retirado da borda do adesivo mostrou crescimento celular anormal na camada externa da pele, confirmando o 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.
Freqüentemente, uma biópsia é realizada porque pode ser semelhante à ceratose actínica ou ao carcinoma de células escamosas.