Keratoderma palmoplantaris transgrediens

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Alternate names

Erythrokeratodermia figurata, congenital familial, in plaques; Erythrokeratodermia variabilis with erythema gyratum repens; Greither disease; Keratosis palmoplantaris transgrediens et progrediens; Greither's disease; Keratosis extremitatum hereditaria progrediens; Transgrediens et progrediens palmoplantar keratoderma

Definition

A rare, isolated, diffuse palmoplantar keratoderma disorder characterized by red-yellow, moderate to severe hyperkeratosis of the palms and soles, extending to the dorsal aspects of the hands, feet and/or wrists and involving the skin over the Achilles' tendon (transgrediens), gradually worsening with age (progrediens) to include patchy hyperkeratosis over the shins, knees, elbows and, sometimes, skin flexures. Hyperhidrosis is usually associated. Histologically, either epidermolytic or nonepidermolytic changes may be seen.

NIH genetic and rare disease info

Keratoderma palmoplantaris transgrediens is a rare disease.


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