Hay-Wells Syndrome

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Hay-Wells syndrome - A case report Síndrome de Hay-Wells - Relato de caso

Hay-Wells syndrome is a rare form of ectodermal dysplasia initially described by Hay and Wells in 1976. It is an autosomal dominant disorder with varying forms of expression featuring congenital abnormalities of the skin, hair, teeth, nails and sweat glands. The present report describes the case of a 17-yearold white boy, the son of nonconsanguineous parents, who presented ankyloblepharon filif...

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Ectodermal Dysplasia Showing the Clinical Overlap between Hay Wells Syndrome and Bowen Armstrong Syndrome

BACKGROUND Several clinical entities combine ectodermal dysplasia (ED) and cleft lip and/or palate (CL/P). These disorders have been recognized with a narrow phenotypic spectrum and very similar clinical features. CASE PRESENTATION We report a case with a clinical diagnosis of Hay Wells syndrome (ankyloblepharon, ED and CL/P), who is a descendent of a mother with Bowen Armstrong syndrome (ED,...

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A FAMILY FROM TURKEY WITH HAY WELLS SYNDROME RESULTING FROM HETEROZYGOUS MUTATION OF P63 GENE Türkiye'deki bir ailede heterozigot p63 mutasyonunun neden olduğu Hay Wells sendromu

INTRODUCTION Hay Wells syndome, also known as ankyloblepharon–ectodermal dysplasia–clefting (AEC) syndrome, is one of the ectodermal dysplasia syndromes. It is an autosomal dominant disorder characterized by findings of ectodermal dyplasia including alopecia, scalp infections, dystrophic nails, hypodontia, ankyloblepharon and cleft lip-palate (1). It was first described by Hay and Wells in 7 in...

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Wells' syndrome.

Eosinophilic cellulitis/Wells' syndrome is a rare dermatosis with erythematous, urticarial plaques that become more indurated and eventually have grey blue discoloration. The histopathology is distinctive, with a diffuse infiltrate composed predominantly of eosinophils but admixed with lymphocytes, histicytes and occasionally multinucleated histiocytes. There is dermal edema with so called "fla...

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Wells Syndrome

A 31-year-old Caucasian female presented to the emergency department (ED) with chief complaint of a gradually progressive right extremity rash for past two days. The patient described a warm, red area over the dorsum of her forearm. The lesion began as a small spot that overnight came to involve most of the arm. The patient, who had similar episodes in the past on her back, stomach, and legs, h...

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ژورنال

عنوان ژورنال: Journal of Nepal Paediatric Society

سال: 2009

ISSN: 1990-7982,1990-7974

DOI: 10.3126/jnps.v29i2.2050