Porokeratosis - Porokeratozahttps://en.wikipedia.org/wiki/Porokeratosis
Porokeratoza (Porokeratosis) je redka motnja keratinizacije. Za porokeratoza (porokeratosis) so značilne kožne lezije, ki se začnejo kot majhne rjavkaste papule, ki se počasi povečujejo in tvorijo nepravilne, obročaste, hiperkeratotične ali bradavicam podobne lezije.

Pogosto se izvaja biopsija, ker je lahko podobna aktinična keratoza ali ploščatoceličnemu karcinomu.

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  • Trdi štrleči robovi so značilni.
    References Porokeratosis 30335323 
    NIH
    Porokeratoza (Porokeratosis) je redka kožna bolezen, za katero so značilne težave s keratinizacijo, kar povzroči dvignjene, obročaste lise ali grobe izbokline na koži. Njegova značilnost pod mikroskopom je prisotnost kornoidne lamela (cornoid lamella), posebne razporeditve celic v zgornji plasti kože. Porokeratoza (Porokeratosis) je na voljo v različnih oblikah, kot je diseminirana površinska aktinska porokeratoza (disseminated superficial actinic porokeratosis), porokeratoza Mibelli (classical porokeratosis of Mibelli), palmarna in plantarna porokeratoza (porokeratosis palmaris plantaris et disseminatum) ter linearna porokeratoza (linear porokeratosis). Pomembno je vedeti, da se lahko porokeratoza (Porokeratosis) potencialno razvije v kožnega raka. Najboljši način za diagnosticiranje porokeratoze (Porokeratosis) je z biopsijo dvignjenega roba, čeprav trenutno ni standardnega protokola zdravljenja.
    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) je bolezen neurejene keratinizacije. To je ena od šestih vrst porokeratoze in običajno prizadene večja območja v primerjavi z drugimi (linearna porokeratoza (linear), porokeratoza Mibelli (Mibelli's), puntata porokeratoza (punctate), palmoplantarna disseminirana porokeratoza (palmoplantar disseminated) in površinska porokeratoza (superficial porokeratosis)). Eruptivni tip porokeratoze je pogosto povezan z rakom, oslabljeno imunostjo ali vnetjem. Dejavniki tveganja vključujejo genetiko, imunsko zatiranje in izpostavljenost soncu. DSAP se začne kot rožnate ali rjave papule (papules) z dvignjenimi robovi na soncu izpostavljenih območjih, ki včasih povzročajo rahlo srbenje. Zdravljenja se razlikujejo in lahko vključujejo lokalne kreme, svetlobno terapijo ali zdravila, kot sta 5-fluorouracil ali retinoidi. Te lezije veljajo za predrakave, z 7,5–10 % možnostjo, da se spremenijo v ploščatocelični (squamous cell) ali bazalnocelični (basal cell) karcinom.
    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
    52-letni moški, prej zdrav, je prišel z plosko, obročasto zaplato na koncu četrtega prsta na nogi, ki je bila tam 2 leti, ne da bi povzročila kakršne koli simptome. Začelo se je kot majhna trda bulica in se je sčasoma povečala navzven. Kljub preizkušanju različnih zdravljenj, kot so krioterapija, kreme, protiglivična zdravila in antibiotiki, se lesija ni izboljšal. Natančen pregled z dermokopijo je pokazal suho, eritematozno sredinsko območje z debelo, grobo obrobo. Majhen košček kože, odvzet z roba lesija, je pokazal nenormalno rast celic v zunanji plasti kože, kar je potrdilo diagnozo porokeratoza Mibelli (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.