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Mibelli's disease I
Also known as:
Mantoux' syndrome
Mibelli's porokeratosis
Porokeratosis of Mibelli
Associated persons:
Charles Mantoux
Vittorio Mibelli
Description:
A rare disease characterised by craterlike keratotic papules of various sizes, with central atrophy and surrounding grooves, involving predominantly the distal parts of the limbs, thighs and perigenital region. The lesions usually begin as small papules of various sizes, which may coalesce in plaques with irregular boundaries, spreading in a centrifugal pattern. The disease, which usually develops early and persists during life, is inherited as an autosomal dominant trait. Both sexes affected; male-to-female ratio 3: 1; onset usually in young adulthood. Autosomal dominant type of inheritance with lower penetrability in females.
Porokeratosis was first described by Isidor Neumann, Edler von Heilwart (1832-1906), in 1875.
Bibliography:
- I. Neumann:
Ueber eine noch wenig bekannte Hautkrankheit (Dermatitis circumscripta herpetiformis).
Vierteljahrsschrift für Dermatologie und Syphilis, Wien, 1875, 2: 41-52.
- V. Mibelli:
Contributo alla studio della ipercheratosi dei canali sudoriferi (porokeratosi).
Giornale italiano di dermatologia e venereologia, Torino, 1893, 28: 313-355.
- C. Mantoux:
Porokératose pokilomateuse palmaire et plantaire.
Annales de dermatologie et de syphiligraphie, Paris, 1903, 4: 15-31.
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