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An excellent response to topical therapy of four congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome patients with an increased concentration of simvastatin ointment

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P < 0.001, respectively; Fig. 1e,f). Overall, HS stage III patients were 5.96 times more likely to be anaemic than HS stage I patients (P < 0.001). The prevalence of… Click to show full abstract

P < 0.001, respectively; Fig. 1e,f). Overall, HS stage III patients were 5.96 times more likely to be anaemic than HS stage I patients (P < 0.001). The prevalence of anaemia (28%) in the AV control cohort was significantly higher than the general population (7.6% of females and 3.5% of males), but consistent with the Bronx population, composed predominantly of African Americans and Hispanics, in whom lower haemoglobin levels are well documented. We also assume a significant component of iron deficiency in this cohort, noting the predominance of women of childbearing age (85.8% of patients were under the age of 40). The correlation of disease severity with degree of anaemia is consistent with our assumption that anaemia in HS patients is attributable to inflammation. We speculate that the significantly lower rate of anaemia in the Danish cohort reflects the fact that this group of patients with self-reported HS likely had less severe disease. Our data also suggest that anaemia in our HS population is multifactorial. While improvement in Hb is expected with treatment of the HS, it remains important to screen for other reversible aetiologies, most notably iron deficiency. Further investigation is warranted concerning the relative contribution of iron deficiency in anaemic HS patients, and in particular discovery of strategies to identify those who might would benefit from iron supplementation. Hepcidin is an interesting candidate in that regard and the subject of ongoing studies at our institution.

Keywords: topical therapy; excellent response; four congenital; response topical; iron deficiency; therapy four

Journal Title: Journal of the European Academy of Dermatology and Venereology
Year Published: 2019

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