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Publikacije (50)

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A. Prohic, A. Selmanagić, N. Bilalović

Epidermolytic hyperkeratosis (EHK) or bullous congenital ichthyosiform erythroderma is a rare autosomal dominant disorder characterized by an early onset, with erythroderma and bullous lesions, leading to severe generalized hyperkeratosis in adulthood. Mutations have been found in keratin 1 and keratin 10 genes. The clinical manifestations of EHK present striking heterogeneity and at least six clinical phenotypes have been identified. We report on a case of EHK in a 12-year-old girl with erythroderma, erosions and blisters on the entire body surface at birth and generalized hyperkeratosis but without severe palm and sole involvement in the later stage. On the basis of clinical and histopathologic findings, the diagnosis of EHK type NPS-3 was made.

Alopecia areata (AA) is a heterogeneous disease characterized by nonscarring hair loss on the scalp or any hair-bearing surface. A wide range of clinical presentations can occur, from a single patch of hair loss to complete loss of hair on the scalp (alopecia totalis, AT) or over the entire body (alopecia universalis, AU). The cause of AA is unknown although most evidence supports the hypothesis that AA is an immunologically mediated disease. The aim of the study was to compare serum levels of total immunoglobulin E (IgE) between patients with AA and healthy subjects, and to assess the difference between the localized form and extensive forms of the disease such as AT and AU. Sixty patients with AA and 50 healthy subjects were enrolled in the study. Fifty patients had localized AA (LAA), and ten patients had AT, AU or AT/AU. Serum levels of IgE were measured using fluoroenzyme immunoassay techniques. Serum levels of total IgE were significantly higher in AA patients than in controls (p<0.05). There was no significant difference in serum levels of total IgE between patients with LAA and those with extensive forms of the disease (p>0.05). The exact role of serum IgE in AA should be additionally investigated in future studies.

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