Harlequin Ichthyosis Treated with Acitretin

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Congenital Ichthyosis: A Case Treated Successfully With Acitretin

INTRODUCTION Lamellar ichthyosis (collodion baby) is a cornification disorder classified under the category of autosomal recessive congenital ichthyosis and characterized by hyperkeratosis. Early-stage retinoid treatment has been shown to improve survival in these patients. In this article, a lamellar ichthyosis case is presented of an infant who had the symptoms at birth and was treated succes...

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Harlequin ichthyosis (or harlequin fetus)

The pat ient was a female neonate bor n prematurely on 36 weeks of gestation by normal vaginal delivery. During pregnancy, mother had no history of fever, drug use or x-ray exposure. The mother was gravida 2 and had no history of abortion. Her first baby was well. On physical examination, the skin was markedly thickened, hard and hyperkeratotic with deep crevices running transversely and vertic...

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A case of harlequin ichthyosis treated with isotretinoin.

Harlequin ichthyosis is a rare congenital ichthyosis classified under the category of Autosomal Recessive Congenital Ichthyoses, which also include lamellar ichthyosis and congenital ichthyosiform erythroderma. It is caused by functional null mutations in the ABCA12 gene, a keratinocyte lipid transporter associated with lamellar granule formation. Patients have a classic clinical presentation a...

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Harlequin ichthyosis: Case report

Harlequin fetus is a rare and the most severe form of the congenital ichthyosis with an autosomal recessive inheritance. Incidence of the disease is nearly 1 in 3,00,000 live births. The disease might be lethal at birth and the affected babies are often premature. Harlequin ichthyosis (HI) is marked by severe keratinized and alligator-like horned skin. The present study reports a new case with ...

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Treatment Of Congenital Ichthyosis With Acitretin

We assessed the clinical efficacy, tolerability and safety of acitretin in a patient with ichthyosis. A newborn infant with ichthyosis who presented at birth with collodion baby appearance, was treated with acitretin. A moderate response to acitretin therapy (1 mg/kg/day) administered for 6 months was observed, with improvement in cutaneous lesions. Clinical improvement was achieved shortly aft...

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ژورنال

عنوان ژورنال: The Journal of Pediatric Research

سال: 2015

ISSN: 2147-9445

DOI: 10.4274/jpr.73792