Hypohidrotic Ectodermal Dysplasia in Three Girls

Year 2007, Volume: 9 Issue: 3, 38 - 40, 01.12.2007

Serpil Sener

Abstract

Hypohidrotic ectodermal dysplasia shows X-linked recessive inheritance in almost all cases. The main features are hypotrichosis, absent or reduced sweating, partial or total anodontia, and a highly characteristic facial physiognomy. Ten percent of female carriers may have partial expression of this disorder. This report presents three girls who have partial expression of hypohidrotic ectodermal dysplasia, because it is rarely seen

Keywords

Hypohidrotic ectodermal dysplasia, female

Üç Kiz Çocukta Hipohidrotik Ektodermal Displazi

Abstract

Hipohidrotik ektodermal displazi olgularin neredeyse tamaminda X’e bagli resesif kalitim göstermektedir. Hastaligin temel bulgulari hipotrikoz, azalmis veya tümüyle kaybolmus terleme, kismi veya tam anodonti ile oldukça karakteristik bir yüz fizyonomisidir. Tasiyici kadinlarin yüzde onunda hastaligin kismi bulgulari mevcut olabilir. Bu makalede hipohidrotik ektodermal displazinin bulgularini kismen gösteren üç kiz olgu nadir görülmesinden dolayi sunulmaktadir

Keywords

Hipohidrotik ektodermal displazi, kadin

References

• Odom RB, James WD, Berger TG. Andrews’ Diseases of the Skin. W.B. 9th ed. W.B. Saunders Co. pp: 722-6, 2000.
• Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC: Dermatology. 2nd ed. Springer-Verlag. pp: 1093-4, 2000.
• Dogan B, Taskapan O, Harmanyeri Y. Bilateral memebasi yoklugu gösteren hipohidrotik ektodermal displazi olgusu. TÜRKDERM. 36:211-2, 2002.
• Cambiaghi S, Restano L, Paakkönen K, Caputo R, Kere J. Clinic al findings in mosaic carriers of hypohidrotic ectodermal dysplasia. Arch Dermatol. 136:217-24, 2000.
• Bartstra HL, Hulsmans RF, Steijlen PM, Ruige M, de Die -Smulders CE, Cassiman JJ. Mosaic expression of hypohidrotic ectodermal dysplasia in an isolated affected female child. Arch Dermatol 130:1421-4, 1994.
• Tüzün Y, Aydemir EH. Genodermatozlar. In: Dermatoloji: Tüzün Y, Kotogyan A, Aydemir EH, Baransü O (eds), Nobel Tip Kitabevleri, Istanbul:577-600. 1994
• Davutoglu M, Gözü A, Fidan M, Dikici B. Hipohidrotik ektodermal displazi (olgu sunumu). KSÜ Tip Fak Derg 3:107-9, 2004.