Porokeratosis - 汗孔角化症
https://en.wikipedia.org/wiki/Porokeratosis
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堅硬且突出的邊緣是其特徵。
relevance score : -100.0%
References
Porokeratosis 30335323 NIH
Porokeratosis 是一種罕見的皮膚疾病,特徵為角化異常,導致皮膚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異常排列,即皮膚表層細胞的特定排列。Porokeratosis 有多種型態,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緣進行活檢,然而目前尚無統一的治療方案。
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.
Disseminated Superficial Actinic Porokeratosis 29083728 NIH
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。與其他類型相比,它通常影響更大的區域(linear、Mibelli's、punctate、palmoplantar disseminated 以及 superficial porokeratosis)。爆發性汗孔角化症常與癌症、免疫力下降或發炎有關。危險因子包括遺傳、免疫抑制和陽光照射。 DSAP 最初會在暴露於陽光的皮膚區域出現粉紅色或棕色的腫塊,邊緣凸起,偶爾伴有輕微的搔癢。治療方法因人而異,可能包括外用乳膏、光療或 5-氟尿嘧啶、類維生素A 等藥物。這些病變被視為癌前病變,約有 7.5-10% 的機會轉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.
Porokeratosis of Mibelli - Case reports 33150040 NIH
一名 52 歲男子,過去身體健康,但在其第四腳趾末端出現一個扁平的環形斑塊,已持續兩年,未引起任何症狀。起初為一小而硬的腫塊,隨時間逐漸向外擴大。雖曾嘗試冷凍療法、外用乳膏、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,發現病灶中心乾燥呈紅色,邊緣厚且粗糙。從病變邊緣切除的一小塊組織顯示表皮異常細胞增生,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.
通常會進行活檢,因為它的外觀類似光化性角化病或鱗狀細胞癌。