Collodion bebek ve konjenital hipotiroidizm birlikteliği: Olgu sunumu
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Date
2007
Authors
Melek, Hamide
Şen, Tolga Altuğ
Köken, Reşit
Demir, Tevfik
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
İktiyozlar, klinik olarak ciltte soyulma ve histopatolojik olarak hiperkeratozisle karekterize olan kornifikasyon bozukluklarıdır. “Collodion” bebek seyrek görülen konjenital bir bozukluk olup, “harlequin” bebekleri andıran hafif dereceli bir patolojiyi ifade etmektedir. “Collodion” bebekler doğumda yağlı parşömen kağıdını andıran, kalın ve gergin bir zarla kaplıdır ve bu tabaka sonradan dökülmektedir. Bu tablo lamellar iktiyozis ve konjenital iktiyoziform eritrodermada da görülmektedir. “Collodion” bebeklerin konjenital anomalilerle veya başka hastalık tablolarıyla birliktelikleri bildirilmiştir. “Collodion” bebeklerin Hirschsprung hastalığı, familyal tiroksin bağlayan globulin fazlalığı, sağırlık, ağlarken asimetrik yüz, ayak parmaklarının distal falankslarının doğuştan yokluğu ve tırnakların normalden daha kalın olması gibi patolojilerle birliktelikleri bildirilmiştir. Konjenital (Harlequin bebek olarak doğmuş olanlarda) ve kazanılmış iktiyotik cilt değişiklikleri olan erişkinlerde daha önce hipotiroidi birlikteliği bildirilmişken, şimdiye kadar sadece bir “collodion” bebekte hipotiroidi birlikteliği bildirilmiştir. Bizim olgumuz bu birlikteliğin olduğu ikinci olgu olacaktır.
Ichthyoses are disorders of cornification characterized clinically by patterns of scaling and histopathologically by hyperkeratosis. Collodion baby is a rare congenital disorder which resembles harlequin fetus, but is milder in severity. Collodion babies are covered at birth by a thick, taut membrane resembling oiled parchment or collodion, which is subsequently shed. This condition is usually seen in lamellar ichthyosis or congenital ichthyosiform erythroderma. There are few reports of collodion baby associated with congenital abnormalities or disease in the literature. Collodion baby associated with asymmetric crying facies, congenital absence of distal phalanges of toes with increased thickness of the finger nails, deafness, Hirschsprung’s disease and familial thyroxin-binding globulin excess have been reported. Hypothyroidism associated with harlequin baby and acquired ichthyosis or ichthyotic skin changes in adulthood have been reported previously but only one collodion baby with congenital hypothyroidism has been reported so far. This report presents the second case in the literature.
Ichthyoses are disorders of cornification characterized clinically by patterns of scaling and histopathologically by hyperkeratosis. Collodion baby is a rare congenital disorder which resembles harlequin fetus, but is milder in severity. Collodion babies are covered at birth by a thick, taut membrane resembling oiled parchment or collodion, which is subsequently shed. This condition is usually seen in lamellar ichthyosis or congenital ichthyosiform erythroderma. There are few reports of collodion baby associated with congenital abnormalities or disease in the literature. Collodion baby associated with asymmetric crying facies, congenital absence of distal phalanges of toes with increased thickness of the finger nails, deafness, Hirschsprung’s disease and familial thyroxin-binding globulin excess have been reported. Hypothyroidism associated with harlequin baby and acquired ichthyosis or ichthyotic skin changes in adulthood have been reported previously but only one collodion baby with congenital hypothyroidism has been reported so far. This report presents the second case in the literature.
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Keywords
Ktiyozis, "Collodion" bebek, Konjenital hipotiroidizm, Ichthyosis, Collodion baby, Congenital hypothyroidism
Citation
Melek, H. vd. (2007). "Collodion bebek ve konjenital hipotiroidizm birlikteliği: Olgu sunumu". Güncel Pediatri, 5(3), 118-120.