Rationale: Sirolimus is a treatment for slow-flow vascular malformations (SFVMs). However, the long-term management remains challenging.
Objectives: The SIROLO study assessed the long-term effects and real-life management of oral sirolimus for SFVMs by investigating data from 15 French tertiary centres for vascular anomalies.
Background: Low-flow malformations (LFMs) are rare diseases with a significant impact on health-related quality of life (HRQoL), especially in children. No disease-specific questionnaire is available for children with LFMs.
Objective: To develop and validate a specific HRQoL questionnaire for children from 11 to 15 years old suffering from LFMs.
Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type (macrocystic LMs, microcystic LMs or both). Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), to provide health professionals with free open access synthesis on optimal management and care of patients with LMs ( https://www.
View Article and Find Full Text PDFAnnular lipoatrophy of the ankle is a rare and unique acquired lipoatrophic panniculitis that mainly affects children. There is no consensus on treatment, and the long-term course is not well known. We present four new pediatric cases that contribute to the understanding of this rare disease.
View Article and Find Full Text PDFWe report 20 newborns who developed, at a median age of 7 days, large abdominal patches of radially arranged purplish telangiectasia in a bilateral and symmetrical pattern in relation to the midline, creating a "butterfly wing" pattern. Clinical examination was normal in 13 newborns, six newborns had abdominal distention, and one newborn had poor weight gain due to inadequate breastfeeding. Most lesions spontaneously resolved within 3 months and did not reoccur for 19 newborns.
View Article and Find Full Text PDFUnlabelled: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genodermatosis with severe blistering. No curative treatment is available. Scientific data indicated that epigallocatechin-3-gallate (EGCG), a green tea extract, might improve the phenotype of RDEB patients.
View Article and Find Full Text PDFBackground: There is limited information regarding early development of soft-tissue and/or bone hypertrophy with facial port-wine stains (PWS).
Objective: We sought to characterize patients with hypertrophic PWS presenting during childhood.
Methods: Patients with a facial PWS and underlying hypertrophy that developed before the age of 18 years were included in a multicenter retrospective study.
Background: Reticular infantile hemangioma (RIH) with minimal or arrested growth (MAG) is an underrecognized variant of infantile hemangioma (IH).
Objective: We describe a new clinical subtype of RIH-MAG associated with lipoatrophy.
Methods: The medical charts and serial clinical photographs and imaging studies of 53 children given a diagnosis between 2004 and 2013 labeled as "abortive," "minimal or arrested growth," "reticular," or "congenital" hemangiomas were reviewed in the departments of dermatology and infantile plastic surgery of 2 French university hospital centers.
Short-term outcomes after percutaneous image-guided cryoablation of symptomatic venous malformations in four consecutive patients (mean age, 42.5 y) are reported. Two patients had local recurrences after previous treatment.
View Article and Find Full Text PDFBackground: Exogenous acne refers to acneiform lesions due to external factors such as cosmetic agents, exposure to various oils, skin rubbing or friction or chloracne, now better called metabolizing acquired dioxin-induced skin hamartoma (MADISH). Here we report a new form of severe inflammatory exogenous acne due to the association of two factors: facial friction with cosmetic agents.
Observations: A 15-, 17- and 19-year-old female presented at the department with severe inflammatory acne.
The case presented here points towards the fact that skin lesion observed with a cowpox virus is a rare event but should be considered more as the number of cases has increased in the last years. Cowpox virus (CPXV) belongs to the Poxviridae family. The transmission of CPXV to humans is caused by wild rodents or mostly by domestic animals and pet rats.
View Article and Find Full Text PDFBackground: Vitiligo often starts in childhood. It is traditionally divided into segmental vitiligo and nonsegmental vitiligo. There are limited data regarding the clinical characteristics of both forms and no comparative study has been performed.
View Article and Find Full Text PDFBackground: Since 1987 we have run a Dermatologic Surgical Paediatric Outpatient Clinic (DSPOC) within the Children's Hospital in Bordeaux.
Objective: We analyse the consequences of an audit survey concerning the management of patients with congenital nevi (CN) seen at this clinic.
Methods: We reviewed the cases of 192 children examined and photographed at the DSPOC during the period January 1990-December 1997.
Capillary malformation-arteriovenous malformation (CM-AVM) is a newly recognized autosomal dominant disorder, caused by mutations in the RASA1 gene in six families. Here we report 42 novel RASA1 mutations and the associated phenotype in 44 families. The penetrance and de novo occurrence were high.
View Article and Find Full Text PDFObjectives: To report the clinical features of the loose anagen hair syndrome and to test the hypothesis that the typical gap between the hair and the inner root sheath may result from hereditary defects in the inner root sheath or the apposed companion layer.
Design: Case series.
Setting: A pediatric dermatology unit (referral center).
Objectives: To assess skin colonization by Malassezia species in full-term healthy newborns, to investigate factors associated with colonization, and to look at acnelike cephalic pustulosis associated with this carriage.
Design: Samples were obtained from neonates and their mothers 0 to 5 days after birth and again 3 weeks later. Clinical patterns of common acnelike pustulosis were reported as mild (<10 papulopustules), moderate (> or =10 papulopustules), or absent.