๐Ÿ—‚ ็ธฝ็›ฎ้Œ„ ๏ฝœ ๐Ÿ“– ่‹ฑๆ–‡ๅŽŸๆ–‡ ๏ฝœ ๐Ÿ“ ๅฎŒๆ•ด็ฟป่ญฏ ๏ฝœ โญ ็ฒพ่ฏ็ญ†่จ˜๏ผˆๆœฌ็ฏ‡๏ผ‰

็–พ็—…ๅฎš็พฉ่ˆ‡ๅˆ†้กž

  • Naegeli-Franceschetti-Jadassohn syndrome ็‚บไธ€็จฎๆฅต็‚บ็ฝ•่ฆ‹็š„ๅค–่ƒšๅฑค็™ผ่‚ฒไธ่‰ฏ (ectodermal dysplasia) ไน‹้บๅ‚ณ่ฎŠ็•ฐๅž‹๏ผŒๅ‘ˆ้ซ”ๆŸ“่‰ฒ้ซ”้กฏๆ€ง้บๅ‚ณ (autosomal dominant)ใ€‚

่‡จๅบŠ็‰นๅพต (Clinical Features)

  • ็‰นๅพต็‚บ้ ธ้ƒจใ€่ป€ๅนน่ˆ‡ๅ››่‚ข็š„็šฎ่†š็ถฒ็‹€ๆ–‘็‰‡็‹€ๅŠๆ–‘้ง็‹€่‰ฒ็ด ๆฒ‰่‘— (reticulate patchy and mottled hyperpigmentation)ใ€‚
  • ็ผบไน็šฎ็ด‹ (absence of dermatoglyphics)ใ€‚
  • ็€ฐๆผซๆ€งๅ’Œ/ๆˆ–้ปž็‹€ๆŽŒ่น ่ง’ๅŒ–็—‡ (diffuse and/or punctate palmoplantar keratoderma)ใ€‚
  • ๆŒ‡็”ฒ็‡Ÿ้คŠไธ่‰ฏ (nail dystrophy)ใ€็‰™้ฝ’็•ฐๅธธ (teeth abnormalities)ใ€‚
  • ๆฑ—่…บๅˆ†ๆณŒไธ่ถณ (hypohidrosis) ๅฐŽ่‡ด่€็†ฑไธ่‰ฏ (heat intolerance)ใ€‚

็—…็จ‹

  • ็ถฒ็‹€่‰ฒ็ด ๆฒ‰่‘—ๅ‚พๅ‘ๆ–ผ้ ธ้ƒจๅ‘จๅœ่ผƒๆ˜Ž้กฏ๏ผŒ้’ๆ˜ฅๆœŸๅพŒ้€ๆผธๆถˆ้€€๏ผŒๆ–ผ่€ๅนดๅฏ่ƒฝๅฎŒๅ…จๆถˆๅคฑใ€‚
  • ็‰™้ฝ’ไพต็Šฏ้€šๅธธๅšด้‡๏ผŒๅฏ่ƒฝๆ–ผ็”Ÿๅ‘ฝๆ—ฉๆœŸๅณๅฎŒๅ…จ่„ซ่ฝใ€‚


ๅœ– 20-33๏ผšNaegeli-Franceschetti-Jadassohn syndrome๏ผŒๅธธ่ฆ‹ๆŽŒ่น ่ง’ๅŒ–็—‡ (palmoplantar keratoderma)ใ€‚