Synonym(s)
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Very rare, hereditary (probably autosomal dominant) keratinization anomaly (genetic defect unknown) with disseminated, comedone-like, follicular keratoses, keratosis palmoplantaris, pachyonychia, onychogryposis and hyperhidrosis. In the mucosal area: lingua plicata, leukoplakia.
S. a. Keratosis follicularis spinulosa decalvans, with a similar clinical symptomatology, in which mutations in the MBTPS2 gene have been detected.
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EtiopathogenesisThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Follicular acneiform hyperkeratoses on the extensor sides of the extremities and the glutaeal and perioral regions, disseminated palmoplantar keratoses with pemphigoid blistering under the plantar calluses, palmoplantar hyperhidrosis, leukoplakia of the oral mucosa, lingua plicata, and nail changes (pachonychia, onychogryposis, scleronychia; oligophrenia).
Differential diagnosisThis section has been translated automatically.
Keratosis follicularis epidemica (has only historical significance)
TherapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
Alfadley A et al (2002) Two brothers with keratosis follicularis spinulosa decalvans. J Am Acad Dermatol 47(Suppl5): S275-278
Aten E et al.(2010) Keratosis follicularis spinulosa decalvans is caused by mutations in MBTPS2. Hum Mutat 31:1125-1133
Azakli HN et al (2014) Keratosis Follicularis Spinulosa Decalvans Associated wıth Leukonychia. West Indian Med J 63: 552-553
- Siemens HW (1929) Keratosis palmo-plantaris striata. Arch Dermatol 157: 392-408.
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Incoming links (8)
Keratosis follicularis congenita; Keratosis follicularis hereditaria gertler; Keratosis follicularis spinulosa decalvans; Keratosis multiformis; Keratosis multiformis idiopathica siemens; Polykeratosis, congenital; Polykeratosis touraine; Siemens syndrome ii;Outgoing links (14)
Acitretin; Hyperhidrosis (overview); Keratosis follicularis epidemica; Keratosis follicularis spinulosa decalvans; Keratosis pilaris; Leukoplakia; Lingua plicata; MBTPS2 Gene; Onychogrypose; Pachyonychia; ... Show allDisclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer