Porokeratosis - 汗孔角化症
堅硬的突出邊緣是其特徵。
relevance score : -100.0%
ReferencesPorokeratosis 是一種罕見的皮膚病，其特徵為角化異常，導致皮膚上出現凸起的環形斑塊或粗糙的腫塊。顯微鏡下的顯著特徵是角質層的存在，這是皮膚表層細胞的特定排列。Porokeratosis 有多種形式，例如 disseminated superficial actinic porokeratosis、classical porokeratosis of Mibelli、porokeratosis palmaris plantaris et disseminatum 以及 linear porokeratosis。值得注意的是，Porokeratosis 可能會發展成皮膚癌。診斷 Porokeratosis 的最佳方法是對凸起邊界進行活檢，儘管目前尚無標準治療方案。
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) 是一種角質化失調疾病，屬於六種汗孔角化症之一。與其他類型（linear、Mibelli's、punctate、palmoplantar disseminated、superficial porokeratosis）相比，DSAP 通常累及較廣泛的皮膚區域。爆發性汗孔角化症常與癌症、免疫功能低下或發炎相關。危險因子包括遺傳、免疫抑制以及陽光曝曬。DSAP 初期在日照部位出現粉紅色或棕色的斑塊，邊緣隆起，偶有輕度瘙癢。治療方式多樣，可能包括外用藥膏、光療或使用 5-fluorouracil (5-氟尿嘧啶)、retinoid (類維生素A) 等藥物。這些病變屬於癌前病變，約有 7.5%–10% 的機會演變為鱗狀細胞癌或基底細胞癌。
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.
一名 52 歲男子，過去身體健康，但其第四腳趾末端出現一個扁平的環形斑塊，已持續兩年且無任何症狀。起初該斑塊為小而硬的腫塊，隨時間逐漸向外擴大。雖曾嘗試冷凍療法、外用藥膏、抗真菌藥物及抗生素等多種治療，病變仍未改善。經皮膚鏡檢查發現斑塊中心乾燥呈紅色，邊緣厚且粗糙。從邊緣取下的一小塊皮膚標本顯示表皮層有異常細胞增生，確診為 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.
通常需要進行活檢，因為其外觀類似光化性角化病或鱗狀細胞癌。