Cases reported "Hypohidrosis"

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1/2. Anhidrotic ectodermal dysplasia with transient hypogammaglobulinemia.

    An 8-month-old white boy with anhidrotic ectodermal dysplasia (AED) who was referred to the north carolina Baptist Hospital because of recurrent respiratory infections and hypogammaglobulinemia is presented. His mother had partial expression of AED suggesting x-linked recessive inheritance in this family. She was incidentally given oral glucocorticoids during pregnancy for the treatment of chronic urticaria. The patient's serum immunoglobulins G, A and M were low at 8 months but normal by 15 months of age, and immunologic evaluation failed to show a defect in antibody production or cell-mediated immunity. Although rare, the diagnosis of AED must be considered in infant boys with recurrent fever and respiratory infections. The diagnostic features of the disease may be subtle in young child prior to the eruption of the characteristic peg-shaped teeth.
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keywords = x-linked
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2/2. Follicular atrophoderma and basal cell carcinomas: the Bazex syndrome.

    Seven members of one family had rare, inherited syndrome of follicular atrophoderma, basal cell carcinomas, and localized anhidrosis. Associated findings in some subjectes were generalized hypohidrosis and "eczema" involving the face shortly after birth. This syndrome shows dominant inheritance, possible x-linked. Follicular atrophoderma is an important skin manifestation, as it has been associated in all reported cases with other systemic or cutaneous abnormalities.
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keywords = x-linked
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