| Abstrakt: |
Three hundred and sixty-six patients with ichthyosis vulgaris were examined, including 229 with Xlinked recessive ichthyosis (X.L.I.) and 137 with autosomal dominant ichthyosis (A.D.I.), among the Jewish population of Israel. The minimal incidence of the disease in this population is about 1: 6500. The disease affected the various ethnic groups differently; among Iraqi Jews there were cases only of X.L.I. compared with only A.D.I. among indian Jews. The disease appeared in both forms In 95% of cases before 5 years of age, and half of the cases showed improvement in the course of time; 80%-95% of the patients noted improvement in the summer months. A family history of "atopy" was found only in about a third of cases of A.D.I. and In none of those with X.L.I. The most pronounced clinical features of the disease were found over the anterior aspect of the legs, and equally in both forms. Follicular keratosis of the arms and hyperkeratosis with prominent creases of the palms and soles were found only in AD.I. The histological findings were diagnostic, especially in those areas where the scaling was obvious, and thus it is recommended to biopsy the anterior shin region. The histology in XL.I. showed compact laminated hyperkeratosis, with thickening of the granular layer; whereas, in A.D.I., the hyperkeratosis was usually of basket-weave type, with a thin or absent granular layer. The sweat glands were normal In structure and number in both forms, while the sebaceous glands were absent only in cases of A.D.I. [ABSTRACT FROM AUTHOR] |
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