عنوان مقاله
نوزاد کولودیون
فهرست مطالب
مقدمه
بیماریزایی
هیستوپاتولوژی
اشکال بالینی بیماری های اغلب مرتبط با نوزاد کولودیون
درمان
بخشی از مقاله
اشکال بالینی بیماری های اغلب مرتبط با نوزاد کولودیون
در مطالعه پیگیری بلند مدت 17 نوزاد کولودیون ، گزارش شده است که بعد از پوست اندازی غشا ظرف مدت 4-1 هفته، 7 مورد ( 41 درصد) به عنوان ایکتیوزیفرم اریتودرمای ارثی، 3 مورد (18 درصد) به عنوان ایکتیوز لاملار ، یک مورد به عنوان سندرم Sjögren-Larsson، یک موردبه عنوانepidermolytic hyperkeratosis ( ایکتیوزیفرم اریتودرما ارثی تاولی) و یک مورد به عنوان بیماری Gaucher مشخص شدند. مابقی بیماران (4 مورد، 24 درصد)، دیگر بیماریهای پوستی را نشان دادند.
کلمات کلیدی:
Collodion Baby Yalçın Tüzün, MD, Aydın İşçimen,* MD, Özer Pehlivan, MD Address: Department of Dermatology Cerrahpaşa Medical Faculty Istanbul University, Fatih, İstanbul, 34098 Turkey E-mail: iscimen_iscimen@yahoo.com * Corresponding author: Aydın İşçimen, MD, Department of Dermatology Cerrahpaşa Medical Faculty Istanbul University, Fatih, İstanbul, 34098 Turkey Published: J Turk Acad Dermatol 2008; 2 (2): 82201r This article is available from: http://www.jtad.org/2008/2/jtad82201r.pdf Key Words: collodion baby, ichthyosis Abstract Background: The term collodion baby is used for newborns in whom all the body surface is covered by thick skin sheets, so called “collodion membrane”. The collodion membrane is the result of an epidermal developmental dysfunction. The collodion membrane is composed of thick skin sheets which resemble translucent, tight parchment paper. In almost all of the collodion membrane cases an autosomal recessive ichthyosiform disease is implicated. Especially, in cases of lamellar ichthyosis, congenital ichthyosiform erythroderma and harlequin ichthyosis frequent association with collodion baby formation has been well documented. Clinically, the collodion babies may encounter dehydration, electrolyte imbalance, temperature malfunction and increasing sepsis risk because of a relatively severe skin damage. Therefore, morbidity and mortality rates are fairly high in these cases. Conclusively, these newborns should be monitored carefully in intense care units and appropriate and supportive treatment must be undertaken. Introduction The term collodion baby refers to a clinic entity used for newborns who are encompassed by a translucent, tight and parchment paper like skin sheets so called collodion membrane, on the entire body surface [1, 2, 3]. Collodion baby as a term was first used by Hallopeau in 1884 [3, 4, 5]. Since then approximately 270 cases were reported [2, 4]. Although, some other diseases and situations may lead to collodion membrane formation in almost all the cases the cause is an autosomal recessive ichthyosiform disease. In order of frequency, congenital ichthyosiform erythroderma (especially, nonbullous form), lamellar ichthyosis and harlequin ichthyosis (which is usually accepted to featuring autosomal recessive inheritance) are responsible. Rarely there may be an association with bullous congenital ichthyosiform erythroderma, Gaucher’s disease and Sjögren-Larsson syndrome. Furthermore, a new form of the disease with an autosomal recessive inheritance called “self healing collodion baby” has been notified where the newborn completely recovers in a couple of weeks. Nevertheless there some other collodion baby causes that have been notified in individual publications [3, 4, 6] (Table 1). Pathogenesis The collodion membrane occurs due to an epidermal cornification disorder just like all the ichthyosiform diseases. Although, the pathogenesis of molecular mechanisms apparently lead to an epidermal cornification
