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Background: Exanthems in children often represent a diagnostic challenge., Purpose: To determine the concordance between pediatric and dermatological diagnoses of exanthems., Design: Prospective study., Procedure: Exanthems that appeared in hospitalized pediatric patients were diagnosed by pediatricians. Pediatric dermatologists, by consensus, either confirmed or modified the diagnoses. Whenever possible, laboratory tests were used to confirm the final clinical diagnoses. Age and evolution were compared with the Kruskal-Wallis test; the kappa coefficient was used to determine concordance., Setting: Institutional tertiary referral pediatric care center., Results: Concordance between pediatric and dermatological diagnoses was found in only 44 patients. When pediatric and dermatological diagnoses of exanthems were classified, the concordance between both diagnoses was very low (kappa = 0.165)., Conclusions: Pediatric specialists, other than dermatologists, failed to diagnose common skin diseases. This may be a consequence of insufficient training in dermatology by medical schools and pediatric residency., (Copyright 2002 S. Karger AG, Basel)

Background: Magnetic resonance imaging (MRI) findings suggestive of neurocutaneous melanosis (NCM) have been reported in asymptomatic patients with giant congenital melanocytic nevi (GCMN)., Objective: To investigate the presence of NCM and the clinical neurologic status of patients with GCMN involving the head an neck., Methods: Thirteen patients with GCMN involving the head and neck were clinically examined by pediatric specialists in dermatology, ophthalmology and neurology. Electroencephalograms, noncontrasted and contrasted computerized tomography (CT) scans and MRI were performed., Results: Eleven of 13 patients with GCMN of the head and neck previously considered asymptomatic were found to present mild but evident neurologic alterations. No signs of NCM were found in the CT scans or in the MRI., Conclusions: Patients with GCMN of the head and neck may have associated neurologic alterations not related to the presence of neurocutaneous melanosis.

8 children with lamellar ichthyosis, 1 with epidermolytic hyperkeratosis and 5 with symmetrical progressive erythrokeratoderma were treated with a new aromatic retinoid (Ro 10-9359). Clinical improvement was dramatic. The children acquired an appearance never obtained before with other managements. The treatment had to be maintained to prevent recurrence. The tolerance to the drug was good. The side-effects were minimal and tended to disappear after several months of treatment. Our results suggest that because of its efficacy, good tolerance and easy administration, the oral retinoid Ro 10-9359 is at present the treatment of choice for the great ichthyotic disorders of children.

During the last 10 years we treated 39 children with severe keratinization disorders with the aromatic retinoid etretinate. Six of these children were followed-up for 8-9 years. Mucocutaneous serum enzymatic and lipid side effects of etretinate were mild, transient and well tolerated. Osseous side effects were present after 4-6 years in all our 6 patients on prolonged retinoid therapy. Asymptomatic osseous neoformation and osseous reabsorption in the absence of calcium, phosphate, and alkaline phosphatase serum alterations have been observed. The growth and development curves and the sexual development of our patients (with exception of a patient with Rud's syndrome) have been normal. Osteoporosis and slender diaphysis were often present at initiation of therapy. On the basis of our findings and recent reports of the literature we suggest restricting retinoid therapy of keratinizing disorders in children to conditions severe enough to be physically, psychologically or socially incapacitating. In an attempt to reduce the risk of chronic toxicity and possibly to allow regression of initial bone alterations, intermittent therapy and combination therapy are recommended.