1958
DOI: 10.1001/archderm.1958.01560060090019
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Report of a Case of Pachyonychia Congenita

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1963
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Cited by 20 publications
(8 citation statements)
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“…The predominant clinical feature of PC is, as the name suggests, a severe dystrophy of the nail. Although there has been much debate about the origin of this lesion (Fleckman, 1985), it is now widely accepted that the nail phenotype in all forms of PC disease arises from hyperkeratosis of the nail bed (Kelly and Pinkus, 1958) and not from alterations in the matrix. In fact, Kelly and Pinkus (1958) reported that the nail plate and matrix appear normal in PC.…”
Section: Discussionmentioning
confidence: 99%
“…The predominant clinical feature of PC is, as the name suggests, a severe dystrophy of the nail. Although there has been much debate about the origin of this lesion (Fleckman, 1985), it is now widely accepted that the nail phenotype in all forms of PC disease arises from hyperkeratosis of the nail bed (Kelly and Pinkus, 1958) and not from alterations in the matrix. In fact, Kelly and Pinkus (1958) reported that the nail plate and matrix appear normal in PC.…”
Section: Discussionmentioning
confidence: 99%
“…The distal nail matrix is hyperplastic and papillomatous, producmg large quantities of homy material mixed with periodic acid-Schiff-positive hyalin. Consequently, the deformed nail resembles a miniature horse's hoof (44).…”
Section: Histopathologic Changesmentioning
confidence: 99%
“…Systemic retinoids, including natural vitamin A (Kelly and Pinkus, 1958), isotretinoin (Thomas et al, 1984), and etretinate (Dupre et al, 1981;Lucker and Steijlen, 1995), have been used to reduce hyperkeratosis. Most patients and physicians find that retinoids are effective in reducing hyperkeratosis, but that they also can increase tenderness and blistering.…”
Section: Resultsmentioning
confidence: 99%