Burden of itch in ichthyosis: a multicentre study in 94 patients.

BACKGROUND: From clinical experience, we know that itch is a major concern for many ichthyosis patients. Nonetheless, no previous studies specifically addressed the issue of itch in ichthyosis. OBJECTIVE: The objective of this study was to specifically address the burden of itch and all its dimensions in ichthyosis patients. METHODS: Ninety-four ichthyosis patients from four different centres were recruited to participate in this cross-sectional, questionnaire-based study. All participants completed the Leuven Itch Scale, a multidimensional self-report instrument that quantifies the frequency, duration, severity, distress, consequences and surface area of itch. RESULTS: Participants included 18 keratinopathic types, 55 autosomal recessive congenital ichthyoses, 11 X-linked recessive ichthyoses (XLRIs), 6 Netherton's ichthyoses, 1 Sjögren-Larsson type, 1 Iocrin ichthyosis and 2 unknown subtypes. Itch occurred in 93% of all patients. In patients with itch, 63% reported that it was often or always present, although most itch episodes were short in duration. Itch, in all its dimensions, was worst in patients with Netherton syndrome. Patients with XLRI had in general a lower itch profile. About half of all ichthyosis patients reported to experience flares during a change in weather, in a hot environment or in stressful situations, whereas a cold environment led to itch in only 26% of patients. The most significant consequences of itching were lesions from scratching, difficulties in falling asleep, bad mood and loss of concentration. CONCLUSIONS: Itch is a major concern in patients with ichthyosis, with significant impact on daily life. Research on future treatments should therefore take itch into consideration and itch should be evaluated in clinical studies. Among the studied subgroups, Netherton patients experienced the most severe consequences.

The effect of synthetic malaria pigment (beta-haematin) on adhesion molecule expression and interleukin-6 production by human endothelial cells.

The effects of synthetic malaria pigment (beta-haematin, BH) on the expression of the intercellular adhesion molecule 1 (ICAM-1) and platelet endothelial cell adhesion molecule 1 (PECAM-1) and the production of interleukin-6 (IL-6) by human microvascular endothelial cells were measured using flow cytometry analysis and immunoenzymatic assay. BH alone did not affect basal levels of ICAM-1, PECAM-1 or IL-6. When added to cell cultures before or with, but not after, lipopolysaccharide or tumour necrosis factor alpha, BH at 1-100 micrograms/mL induced a dose-dependent inhibition of ICAM-1 and PECAM-1 expression and IL-6 production. Cell viability and human leucocyte antigen A,B,C expression remained unaffected. Similar, though more variable, results were obtained using human umbilical vein endothelial cells. These results suggested that accumulation of pigment within endothelial cells following repeated malaria infection reduces local inflammation and parasite sequestration through inhibition of either cytokine production or parasitized erythrocyte receptors on endothelial cells.