Three cutaneous manifestations are characteristic of Bart syndrome: congenital localized absence of skin (CLAS), mucocutaneous blistering, and nail abnormalities. Six cases of Bart syndrome are herein reported. Localized absence of skin is present at birth, particularly on the anterior aspects of the lower extremities and dorsa of the feet. Physical trauma in utero has been proposed as a mechanism to explain the denuded areas on the limbs. The recurrent, highly similar pattern of the congenital defect in regard to location and clinical appearance in our patients and in most of the reported cases strongly suggests that trauma is too simplistic an explanation. Because of the observed bilateral and symmetric distribution of denuded areas in an S-shaped broad band, their sharply demarcated borders, the involvement of the toe webs, and the frequent similar involvement of the soles, we suggest that congenital localized absence of skin in Bart syndrome may follow the lines of Blaschko.

Blister↗

PubMedDate not supplied

Prevalence and nature of nail alterations in pediatric patients.

The purpose of this investigation was to explore the frequency and nature of ungual alterations in patients of a pediatric dermatology department at a third-level pediatric hospital. The first 20 patients with nail alterations seen each year during a 5-year period from 1992 through 1996 were included, totaling 100 patients. The rate of nail alterations was 11% (1/9) in pediatric dermatology patients. There were 5 infants, 19 preschoolers (2- to 5-year-olds), 38 school children (6- to 11-year-olds), and 38 adolescents (12- to 17-year-olds). The most frequent diagnoses were onychomycosis (23), nail alterations in a genodermatosis (23), nail alterations associated with dermatoses (16), onychocryptosis (11), and paronychia (10). Toenails were involved in 54 patients, fingernails in 25, and both in 21 patients. Twenty nails were involved in 21 patients. A high prevalence of nail alterations was found in pediatric dermatology patients, some of which were nonspecific, while others provided important diagnostic clues.

Adolescent↗

PubMedDate not supplied

Treatment of 100 children with papular urticaria with thiamine chloride.

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Child↗