HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, irregularly autosomal-dominantly inherited ((POROK1; OMIN:175800), multifocal differentiation disorder of the epidermis with parakeratosis in the middle of hyperkeratosis, which belongs to the porokeratoses (naming: poros = opening; keras = horn; incorrect designation, because the cornification anomaly does not originate from the follicular epithelium or the acrosyringium).
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ManifestationThis section has been translated automatically.
Mainly occurring during childhood and adolescence, but also in later adulthood.
Men are affected 2x (3x) as frequently as women.
Apparently, however, families with more than 1 variant of a porokeratosis type exist, so that a strict separation must be questioned (Sybert).
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Clinical featuresThis section has been translated automatically.
Mostly single or few (rarely multiple: differentiation from the disseminated type is difficult!), rapidly growing, brownish keratotic papules and plaques.
Initial: 0.1-0.2 cm large, mostly solitary, yellowish, yellowish or reddish-brown, asymptomatic papules with an adherent, non-detachable central horn spine. In case of permanent lateral pressure (e.g. by footwear) the horn papules can become painful. By gradual growth of area and thickness (random) confluence of the individual papules. This results in roundish, circine or garland-shaped plaques up to 0.5-10.0 cm in size (or even larger) with a verrucous or slightly atrophic surface. Accompanying symptoms: Nail deformities.
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Note(s)This section has been translated automatically.
In multiple flocks, porokeratosis superficialis disseminata actinica should be considered in the first instance.
LiteratureThis section has been translated automatically.
- Agarwal S, Berth-Jones J (2002) Porokeratosis of Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol 146: 338-339
- Ehlers G et al (1971) Porokeratosis Mibelli with multiple precanceroses and squamous cell carcinomas. Dermatologist 22: 68-73c
- Judge MR et al (1990) Disseminated porokeratosis in an infant with craniosynostosis. Br J Dermatol 123: 249-254
- Mibelli V (1893) Contributo alla studio della ipercheratosi dei canali sudoriferi (porokeratosi). G Ital Dermatol Venereol (Torino) 28: 313-355
- Sybert VP (2010) Genetic Skin Disorders (end ed.).Oxford University Press, S 89-92
- Wallner JS et al (2003) Verrucous porokeratosis of Mibelli on the buttocks mimicking psoriasis. Cutis 72: 391-393
- Weidner T et al (2017) Treatment of Porokeratosis: A Systematic Review. At J Clin Dermatol 8:435-449
Incoming links (20)Hyperkeratosis concentrica; Hyperkeratosis figurata centrifugata atrophicans; Hyperkeratosis follicularis et parafollicularis in cutem penetrans; Interface dermatitis; Keratoatrophic nevus; Keratoatrophodermia; Keratoatrophodermia hereditaria chronica et progressiva; Keratodermia excentrica; Mibell's disease; Parakeratosis; ... Show all
Outgoing links (27)Acitretin; Actinic keratosis; Alopecia areata (overview); Cryosurgery; Dermis; Elastosis perforans serpiginosa; Electrocoagulation; Granuloma anulare classic type; Hyperkeratoses; Imiquimod; ... Show all
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