Palmoplantar keratoderma, punctate type 2 Q82.8

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).

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).

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

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.

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3. Itin PH (1995) Porokeratosis plantaris, palmaris et disseminata with multiple filiform hyperkeratosis and nail dystrophy. dermatologist 46: 896-872
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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