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Palmoplantar keratoderma, punctate type 2Q82.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 23.05.2023

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Synonym(s)

MIM 175860; Porokeratosis plantaris palmaris et disseminata; Porokeratosis plantaris palmaris et plantaris; Spiky palmoplantar keratosis

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HistoryThis section has been translated automatically.

Guss, Osbourn and Lutzner, 1971

DefinitionThis section has been translated automatically.

Rare genodermatosis with primary manifestation on palmae and plantae and later dissemination, initially assigned to porokeratoses based on histologic features (evidence of a plume-like parakeratosis).

EtiopathogenesisThis section has been translated automatically.

Autosomal dominant mutation in the MVD gene (Jägle S et al.2021). The mutated gene is located on chromosome 17q24 (Stevens HP et al.1996).

LocalizationThis section has been translated automatically.

Located on the trunk and extremities, especially palmoplantar.

Clinical featuresThis section has been translated automatically.

Symmetrically distributed, approximately 0.2-0.4 cm large keratotic papules with a tendency to polycyclic confluence.

HistologyThis section has been translated automatically.

Hyperkeratosis, acanthosis, columnar parahyperkeratosis (cornoid lamella).

Differential diagnosisThis section has been translated automatically.

Porokeratosis Mibelli; Porokeratosis superficialis disseminata actinica.

External therapyThis section has been translated automatically.

The treatment of the horny papules is carried out with keratolytic externals, e.g. with 2-5% salicylic acid (Salicylvaseline Lichtenstein, R228, R227 ), 5-10% urea, 3% NaCl R105 or 5% lactic acid R108.

In pressure-exposed areas, surgical procedure if necessary, either by curettage or laser treatment (Erbium-YAG or CO2 laser).

Internal therapy with acitretin is not very promising according to our own experience.

Internal therapyThis section has been translated automatically.

Test with acitretin (neotigason) 0,5-1,0 mg/kg bw/day.

Progression/forecastThis section has been translated automatically.

Development of carcinomas is possible.

LiteratureThis section has been translated automatically.

  1. Brasch J et al (1985) Porokeratosis plantaris, palmaris et disseminata. Dermatologist 36: 459-461
  2. Cast SB, Osbourn RA, Lutzner MA (1971) Porokeratosis plantaris, palmaris, et disseminata: a third type of porokeratosis. Arch Derm 104: 366-373
  3. Itin PH (1995) Porokeratosis plantaris, palmaris et disseminata with multiple filiform hyperkeratosis and nail dystrophy. dermatologist 46: 896-872
  4. Lanka P et al (2015) Punctate Porokeratosis Palmaris et Plantaris.Indian J Dermatol 60:284-286.
  5. Shaw JC et al (1984) Porokeratosis plantaris palmaris et disseminata. J Am Acad Dermatol 11: 454-460
  6. Wei SC et al (2003) Identification of a locus for porokeratosis palmaris et plantaris disseminata to a 6.9-cM region at chromosomes 12q24.1-24.2 Br J Dermatol 149: 261-267

Authors

Last updated on: 23.05.2023

Author: Prof. Dr. med. Peter Altmeyer