Greithers sykdom
Engelsk navn: Transgrediens et progrediens palmoplantar keratoderma
Engelske synonym: Greither disease,Keratosis extremitatum hereditaria progrediens,Keratosis palmoplantaris transgrediens et progrediens,Progressive diffuse PPK,Progressive diffuse palmoplantar keratoderma,Transgrediens et progrediens PPK
Definisjon
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.
Fra Orphanet
ORPHA: 495
Klassifiseringsnivå: Disorder
ICD-10: Q82.8
