ABSTRACT
Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations.
Subject(s)
Autoimmune Diseases/complications , Fever/complications , Inflammation/complications , Skin Diseases/etiology , Child , Decision Trees , Humans , Recurrence , Skin Diseases/immunologyABSTRACT
A method for analysing traces of nitrites is described. The method is based on the diazotization of a heteroaromatic primary amine-4-methyl-7-aminocoumarin (Coumarin 120((R)))-by nitrite ions in acidic medium. A large excess of Coumarin 120((R)) is mandatory for a quantitative reaction. The corresponding in-situ-produced diazonium salt is stable at room temperature and can be selectively isolated in the aqueous layer by extraction of the excess of unreacted Coumarin 120((R)) with ethyl acetate. The hydrolysis of the diazonium salt at 100 degrees C yields a fluorescent product, 4-methyl-7-hydroxycoumarin (4-methylumbelliferone), which is measured by direct spectrofluorimetry (lambda(ex) = 325 nm; lambda(em) = 380 nm). In this way levels of nitrites in water samples as low as 10 mug 1(-1) can be easily quantified.
