Porokeratosis - Porokeratóza
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References
Porokeratosis je zriedkavé kožné ochorenie charakterizované problémami s keratinizáciou, čo má za následok vyvýšené prstencové škvrny alebo drsné hrbolčeky na koži. Jeho charakteristickým znakom pod mikroskopom je prítomnosť koroidnej lamely, špecifického usporiadania buniek v hornej vrstve kože. Porokeratosis prichádza v rôznych formách, napríklad disseminated superficial actinic porokeratosis, classical porokeratosis of Mibelli, porokeratosis palmaris plantaris et disseminatum, and linear porokeratosis. Je dôležité poznamenať, že porokeratosis sa môže potenciálne rozvinúť do rakoviny kože. Najlepší spôsob, ako diagnostikovať porokeratosis , je biopsia zvýšenej hranice, aj keď v súčasnosti neexistuje štandardný liečebný protokol.
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) je ochorenie narušenej keratinizácie. Je to jeden zo šiestich typov porokeratózy a zvyčajne postihuje väčšie oblasti v porovnaní s ostatnými (linear, Mibelli's, punctate, palmoplantar disseminated, and superficial porokeratosis) . Erupčný typ porokeratózy často súvisí s rakovinou, oslabenou imunitou alebo zápalom. Rizikové faktory zahŕňajú genetiku, potlačenie imunity a vystavenie slnku. DSAP začína ako ružové alebo hnedé hrbolčeky so zvýšenými okrajmi v oblastiach vystavených slnku, ktoré niekedy spôsobujú mierne svrbenie. Liečba sa líši a môže zahŕňať lokálne krémy, svetelnú terapiu alebo lieky ako 5-fluóruracil alebo retinoidy. Tieto lézie sa považujú za prekancerózne, s 7. 5 - 10 % pravdepodobnosťou, že sa zmenia na spinocelulárny alebo bazalióm.
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.
52-ročný muž, predtým zdravý, prišiel s plochou prstencovou náplasťou na konci štvrtého prsta na nohe, ktorá tam bola 2 roky bez akýchkoľvek príznakov. Začalo to ako malý, tvrdý hrbolček a časom sa to rozrástlo. Napriek skúšaniu rôznych spôsobov liečby, ako je kryoterapia, krémy, antimykotiká a antibiotiká, náplasť sa nezlepšila. Pri bližšom skúmaní pomocou dermokopie sa ukázal suchý červený stred s hrubým hrubým okrajom. Malý kúsok kože odobratý z okraja náplasti vykazoval abnormálny rast buniek vo vonkajšej vrstve kože, čo potvrdilo diagnózu 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.
Často sa vykonáva biopsia, pretože môže vyzerať podobne ako aktinická keratóza alebo spinocelulárny karcinóm.