The British journal of dermatology
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Bullous ichthyosiform erythroderma (BIE) and non-bullous ichthyosiform erythroderma (NBIE) are rare congenital ichthyoses. Generalized pustular psoriasis (GPP) of von Zumbusch type is a rare and severe form of psoriasis marked by desquamative and pustular erythroderma associated with fever and altered general conditions. We report two adults with an ichthyosis typical of BIE in the first case and NBIE in the second, without any previous history of psoriasis, who presented with a severe and relapsing GPP of von Zumbusch type. Using current knowledge of the genetic relationship between psoriasis and congenital ichthyoses, we discuss the possibility of a common physiopathological link between congenital ichthyoses and GPP, and examine the possible therapeutic problems resulting from this pathological association, especially in BIE.
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Clinical Trial
Plasma concentrations of lignocaine and prilocaine after a 24-h application of analgesic cream (EMLA) to leg ulcers.
Although the lignocaine (lidocaine)-prilocaine cream EMLA has been extensively studied for the relief of acute treatment-related pain from sharp leg ulcer debridement, no data exist on systemic absorption from prolonged application in patients with chronically painful ulcers. ⋯ The results indicate that a 24-h application of 5-10 g EMLA results in peak plasma concentrations of the two local anaesthetics, which combined are less than one-fifth of those associated with toxic reactions. The analgesic efficacy of EMLA for the relief of chronic ulcer pain deserves further study.
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Multicenter Study
Evaluation of diagnostic criteria for atopic dermatitis: validity of the criteria of Williams et al. in a hospital-based setting.
Surveys of the prevalence of atopic dermatitis (AD) have been carried out world-wide, but the results vary widely. The differences probably result from the use of different diagnostic criteria. Williams et al. proposed minimum, simplified, diagnostic criteria that require no invasive test and are easy to use. Pilot studies in European countries showed their suitability for implementation both in hospitals and in the community, and their high sensitivity and specificity. ⋯ The diagnostic efficiency of the criteria of Williams et al. was basically similar to those of Hanifin and Rajka and of Kang and Tian in our out-patient settings. However, those of Williams et al. were easier to apply and required no invasive tests.
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Congenital ichthyoses are a group of heterogeneous disorders of cornification. Autosomal recessive congenital ichthyosis (ARCI) can be clinically subdivided into congenital ichthyosiform erythroderma and lamellar ichthyosis. Ultrastructurally, ARCI is classified into four groups: ichthyosis congenita (IC) types I-IV. ⋯ None of the patients carried the transglutaminase 1 mutation. We conclude that ultrastructural findings resembling those detected in previous HI cases (type 1 and 2) can also be found in patients who do not have classic clinical features of that rare ichthyosis. This may be due to lack of specificity of ultrastructural markers for HI or to its clinical heterogeneity.