Netherton syndrome associated with idiopathic congenital hemihypertrophy.
Netherton syndrome is a rare genodermatosis comprised of anichthyosiform dermatitis, hair shaft defects, and atopic features. Other problems associated with Netherton syndrome are delayed growth and development, immune abnormalities, recurrent infections, and intermittent aminoaciduria. We describe an 18-month-old girl with Netherton syndrome who had idiopathic congenital hemihypertrophy on her right side with contralateral benign nephromegaly in addition to the characteristic clinical signs of the syndrome. To our knowledge, this is the first case of Netherton syndrome associated with idiopathic congenital hemihypertrophy to be reported.
Yerebakan
Ozlem
O
Departments of Dermatology, Pediatric Immunology, Medical Genetics, and Pathology, Akdeniz University School of Medicine, Antalya, Turkey. yerebaka@med.akdeniz.edu.tr
Uğuz
Ayşen
A
Keser
Ibrahim
I
Lüleci
Güven
G
Ciftçioğlu
Mehmet Akif
MA
Başaran
Erdal
E
Alpsoy
Erkan
E
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001707:Biopsy, Needle; D001842:Bone and Bones / Q000002:abnormalities*; D003876:Dermatitis, Atopic / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D002658:Developmental Disabilities / Q000175:diagnosis; D005260:Female; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D016113:Ichthyosiform Erythroderma, Congenital / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D007150:Immunohistochemistry; D007223:Infant; D011379:Prognosis; D018570:Risk Assessment; D012536:Scalp Dermatoses / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D012868:Skin Abnormalities / Q000175:diagnosis*; D013577:Syndrome