Results: On average, the etiological diagnosis was established 11 months after the onset of erythroderma. The underlying causes observed included immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton syndrome (18%), and eczematous or papulosquamous dermatitis (20%). Five patients (10%) had erythroderma of unknown origin. The following parameters were of value in determining the ...

Netherton syndrome (NS) is a rare autosomal recessive disorder, characterized by a classical triad of clinical features, including congenital ichthyosiform erythroderma, trichorrhexis invaginata, and atopic diathesis coupled with frequent bacterial infections (1). The genetic basis for the disease has been recently identified with mutations in gene SPINK5, which is involved in the regulation of...

We study the role of banks in creating inside money. We show that, even in the absence of asymmetric information or an agency problem, the private provision of inside money is inefficient. The reason is that inside money affects prices and the welfare of non-bank customers, and banks do not internalize this. Under competition, banks tend to generate too much money relative to what is socially e...

The immunologic abnormalities in patients with allergic skin diseases occur as a consequence of primary defects residing within the epidermis, suggesting an association between skin barrier dysfunction and immune abnormalities (1). The skin barrier is located in the uppermost layers of the epidermis, the stratum corneum, with important elements: in-tracellular keratin filaments, intercellular l...

The Supreme Court’s trilogy of evidence cases, Daubert, Joiner, and Kumho Tire appear to mark a significant departure in the way scientific and expert evidence is handled in federal court. By focusing on the underlying methods used to generate the experts’ conclusions, Daubert has the potential to impose a more rigorous standard on experts. Given this potential, some individuals have called for...

48 Pharmaceutical Technology FEBRUARY 2005 S. Klick, PhD, is a team manager, Analytical Development, at AstraZeneca R&D (Mölndal Sweden), tel. 146 31 7761758, fax 146 31 7767337, [email protected]. P.G. Muijselaar, PhD, is a senior analytical scientist and T.K. Gerding, PhD, is a manager, Sector Chemical & Pharmaceutical Development, at Solvay Pharmaceuticals (The Netherlands). J. Wat...

M ost statistics on the incidence of hereditary ichthyosis are acknowledged as best-guess estimates. The exceptions are population-based studies for the prevalence of steroid sulfatase deficiency (xlinked ichthyosis) and mutations in filaggrin (ichthyosis vulgaris). We wondered whether the unique International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code for...

harlequin ichthyosis is a rare and the most severe form of congenital ichthyosis. although prenatal diagnosis is difficult for this disorder, recently, this obstacle has markedly improved with the use of dna-based prenatal diagnosis. here in, we presented a neonate with harlequin ichthyosis born by assisted reproductive technology (art). in this case, the diagnosis of harlequin ichthyosis was n...

IN the complete form of this familial syndrome males suffer from corneal dystrophy, congenital ichthyosis (ichthyosis simplex), and allergic manifestations such as asthma and hay fever. The complete syndrome was noted in two patients. Other male members had corneal dystrophy and allergy, corneal dystrophy and ichthyosis, ichthyosis alone, ichthyosis and allergy, or asthma (Fig. 1). Except for t...