Porokeratosis - 汗孔角化症
■硬く突き出たエッジが特徴的。
References
Porokeratosis は、角質化の問題を特徴とするまれな皮膚疾患で、皮膚に隆起したリング状の斑点や粗い隆起が生じます。顕微鏡下でのその特徴は、皮膚の最上層にある細胞の特定の配置である角状層の存在です。 Porokeratosis には disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, and linear porokeratosis などさまざまな形式があります。 porokeratosis は皮膚がんに発展する可能性があることに注意することが重要です。 porokeratosis を診断する最良の方法は、隆起した境界の生検を行うことですが、現在、標準的な治療プロトコルはありません。
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) は角化障害の病気です。これは 6 つのタイプの孔角化症のうちの 1 つであり、通常、他のものと比較してより広い範囲に影響を及ぼします (linear, Mibelli's, punctate, palmoplantar disseminated, and superficial porokeratosis) 。噴出型の汗孔角化症は、多くの場合、がん、免疫力の低下、または炎症に関連しています。危険因子には、遺伝学、免疫抑制、日光曝露が含まれます。 DSAP は、日光にさらされた領域の端が盛り上がったピンクまたは茶色の隆起として始まり、わずかなかゆみを引き起こす場合があります。治療法はさまざまで、局所クリーム、光線療法、5-フルオロウラシルやレチノイドなどの薬物療法が含まれる場合があります。これらの病変は前がん性であると考えられており、 7. 5 - 10 % の確率で扁平上皮がんまたは基底細胞がんに変化します。
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.
以前は健康だった52歳の男性が、第4指の端に平らなリング状の斑点を抱えて来院しました。この斑点は2年間存在していましたが、何の症状も引き起こしていませんでした。最初は小さくて硬い隆起でしたが、時間の経過とともに外側に大きくなっていきました。凍結療法、クリーム、抗真菌薬、抗生物質などのさまざまな治療法を試したにもかかわらず、パッチは改善しませんでした。ダーモコピーで詳しく調べると、中央が乾燥して赤く、厚くて粗い境界があることがわかりました。パッチの端から採取した小さな皮膚片には、皮膚の外層に異常な細胞増殖が見られ、 porokeratosis of Mibelli の診断が確認されました。
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.
日光角化症や扁平上皮癌に似ている可能性があるため、生検が行われることがよくあります。