Palmoplantar keratoderma, punctate type 2 Q82.8

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

History
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Guss, Osbourn and Lutzner, 1971

Definition
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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).

Etiopathogenesis
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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).

Localization
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Located on the trunk and extremities, especially palmoplantar.

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

Histology
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Hyperkeratosis, acanthosis, columnar parahyperkeratosis (cornoid lamella).

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

Progression/forecast
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Development of carcinomas is possible.

Literature
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  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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 23.05.2023