A syndrome of marked acanthosis, irregular hyperkeratosis and parakeratosis and perivascular infiltration. It is characterised by redness of palms and soles, followed by scaling and thickening, localised or diffuse, extending progressively to dorsal surface. Erythema remains. Hyperhidrosis is frequently associated; frequently, eczematisation; continuous patchiform progression of new lesions on extremities. Poor physical development and mental retardation. Progresses through life. Onset in first month of life, both sexes affected. Inheritance is autosomal recessive.
Papillon-Lefèvre syndrome, under Paul-Henri Papillon, French dermatologist.
Greither's keratosis, under Aloys Greither, German dermatologist, 1914-1986.
- I. Neumann:
Ueber das keratoma hereditaria.
Archiv für Dermatologie und Syphilis, Berlin, 1898, 42: 163-174.
- F. Kogoj:
Die Krankheit von Mljet ("Mal de Meleda").
Acta dermato-venereologica, Stockholm, 1934, 15: 264-299.
- A. Franceschetti, V. Peinhart, U.N. Schnyder:
La maladie de Meleda.
Journal de génétique humaine, 1972, 20: 267-296.