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Syndrome in question. Hay-Wells syndrome.

Tonolli VM, Stolf HO, Tonello CS, Pires RB, Abbade LP - An Bras Dermatol (2014 Mar-Apr)

Bottom Line: Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder.The treatment is aimed to prevent clinical complications.We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon.

View Article: PubMed Central - PubMed

Affiliation: "Júlio de Mesquita Filho" Paulista State University, Botucatu, SP, Brasil.

ABSTRACT
Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent clinical complications. We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon. The importance of the correct diagnosis is emphasized, besides the investigation of the associated diseases, treatment of complications and genetic counseling of the parents.

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Related in: MedlinePlus

Ungual dystrophy in hands and feet
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f03: Ungual dystrophy in hands and feet

Mentions: A four-month old male patient, born from non-consanguineous parents, coming from thecountryside of São Paulo, was referred to our clinic due to chronic diarrhea,protein-energy malnutrition and skin lesions. At admission, he presented exulcerationscovered with honey-colored hematic crusts on the anterior chest, upper limbs, dorsalarea, face and especially on the scalp. The patient also had a cleft palate, deformityof the ears, decreased amount of eyelashes and eyebrows, besides erythematous papules onlower limbs, micropenis, dystrophy on all nails and personal history of surgery forankyloblepharon (Figures 1-3). Skin biopsy result suggested epidermolysis bullosa.


Syndrome in question. Hay-Wells syndrome.

Tonolli VM, Stolf HO, Tonello CS, Pires RB, Abbade LP - An Bras Dermatol (2014 Mar-Apr)

Ungual dystrophy in hands and feet
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4008080&req=5

f03: Ungual dystrophy in hands and feet
Mentions: A four-month old male patient, born from non-consanguineous parents, coming from thecountryside of São Paulo, was referred to our clinic due to chronic diarrhea,protein-energy malnutrition and skin lesions. At admission, he presented exulcerationscovered with honey-colored hematic crusts on the anterior chest, upper limbs, dorsalarea, face and especially on the scalp. The patient also had a cleft palate, deformityof the ears, decreased amount of eyelashes and eyebrows, besides erythematous papules onlower limbs, micropenis, dystrophy on all nails and personal history of surgery forankyloblepharon (Figures 1-3). Skin biopsy result suggested epidermolysis bullosa.

Bottom Line: Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder.The treatment is aimed to prevent clinical complications.We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon.

View Article: PubMed Central - PubMed

Affiliation: "Júlio de Mesquita Filho" Paulista State University, Botucatu, SP, Brasil.

ABSTRACT
Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent clinical complications. We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon. The importance of the correct diagnosis is emphasized, besides the investigation of the associated diseases, treatment of complications and genetic counseling of the parents.

Show MeSH
Related in: MedlinePlus