Dermatologic features of Smith-Magenis syndrome.

Smith-Magenis syndrome (SMS) is characterized by distinctive facial and skeletal features, developmental delay, cognitive impairment, and behavioral abnormalities, including self-injurious behaviors. We aimed to investigate whether cutaneous features are common in SMS. We performed a complete skin examination in 20 young SMS patients. Skin features secondary to self-injurious behavior, such as bites, abrasions, dystrophic scars, limited spots of hyperkeratosis, anomalies of the nails, and whitlows, were found in the majority of patients. Acral pachydermia and fissured plantar keratoderma were common. Xerosis was constant and associated with extensive keratosis pilaris in the majority of patients. Dermatofibromas were frequent in older patients. The hair was dense and shiny, with an unusual hairline. Eyelash trichomegaly and heavy brows were common, as well as folliculitis on the back. The skin features of SMS have rarely been reported in the literature. Some of these are the consequence of neurobehavioral features, but some cutaneous features and abnormalities of appendages have not been reported in other related syndromes. Skin manifestations of SMS are varied, sometimes induced by self-injurious behavior and sometimes more specific. It remains to be determined whether the combination of the two kinds of signs could contribute to early diagnosis of the syndrome.

Clinical phenotype of scabies by age.

OBJECTIVE: Scabies has a clinical presentation that seems to vary according to age. We conducted a prospective study with the goal of delineating the clinical presentation of the disease into 3 groups of age: infants, <2 years; children, 2 to 15 years; and adults, >15 years. METHODS: This trial was a prospective, multicenter observational study in consecutive patients with a confirmed diagnosis of scabies who were seen in 13 French Departments of Dermatology and Pediatric Dermatology between April 2010 and April 2011. A standardized questionnaire was completed for each patient. To identify factors associated with patient age, comparisons between the 3 age groups were conducted by using univariate and multivariate multinomial logistic regression analyses. RESULTS: A total of 323 individuals were included; the gender ratio (female:male) was 1.2:1. In univariate analysis, infants were more likely to have facial involvement. In multivariate logistic regression, relapse was more frequent in children (odds ratio [OR]: 2.45 [95% confidence interval (CI):1.23-4.88]) and infants (OR: 3.26 [95% CI: 1.38-7.71]). In addition, family members with itch (OR: 2.47 [95% CI: 1.04-5.89]), plantar (OR: 20.57 [95% CI: 7.22-58.60]), and scalp (OR: 16.94 [95% CI: 3.70-77.51]) involvement were also found to be independently associated with the age group <2 years. CONCLUSIONS: There is a specific clinical presentation of scabies in infants and children. Taking into account these specificities may be helpful for the early diagnosis and the identification of cases to prevent the propagation of the disease.