Medial fronto-facial capillary malformations.

OBJECTIVE: To evaluate the characteristics of facial medial capillary malformations (CM), which differ from salmon patches by their wider extent, darker color, and incomplete resolution. STUDY DESIGN: Children were prospectively recruited from pediatric dermatology clinics and retrospectively from clinical and photographic databases. RESULTS: From June 2006 to June 2008, 84 children (56 girls; 66.6%) were included. The medial fronto-FCM (FFCM) involved the forehead and glabella (100%), upper eyelids (57.1%), nose (66.6%), philtrum (50.0%), and upper lip (22.6%). Extended forms were observed in 26.2%. A similar FFCM was observed within the family in 27.3% of cases. Outcome data showed complete regression in 10%, incomplete in 71.1%, and unchanging in 18%. An association with an extra facial CM was found 67.8%. Nape and/or occipital CM were associated in 63.8%. A median dorsal CM, mostly lumbosacral, was observed in 13.4%. An associated disease was seen in 33.3%. Neurological anomalies were observed in 9.5% (two cases of developmental delay, two of epilepsy, one of macrocephaly, one of cerebral arteriovenous malformation, one of cutis marmorata telangiectatica congenita, one of "macrocephaly- cutis marmorata telangiectatica congenita," and one of Rubinstein Taybi syndrome). No correlation between the site or the extent of the FFCM and extrafacial vascular or neurological anomaly was found. CONCLUSIONS: This study identifies a specific type of congenital medial FFCM that looks like salmon patch but has a wider median topography, a darker color, with slower and often incomplete resolution. Family cases are often observed. Despite their slow and incomplete regression, the aesthetic consequences are mild.

Segmental and nonsegmental childhood vitiligo has distinct clinical characteristics: a prospective observational study.

BACKGROUND: 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. OBJECTIVE: To compare the clinical features of nonsegmental and segmental vitiligo in children. PATIENTS AND METHODS: We performed a prospective observational study. Consecutive children with vitiligo seen between October 2005 and December 2007 in the 11 French Departments of Pediatric Dermatology were included. A standardized evaluation was completed after total body clinical examination. A second examination was performed 1 year after inclusion. The clinical characteristics of segmental vitiligo and nonsegmental vitiligo were compared. RESULTS: A total of 114 children with vitiligo were included. Compared with segmental vitiligo, nonsegmental vitiligo was associated with a higher number of lesions (more than 5 patches in 65.17% vs 20% of patients, P < .0001) and a larger body surface area of involvement (9.8% +/- 2.51% vs 3.48% +/- 1.6%, P +/- .01). A higher incidence of the Koebner phenomenon (47.19% vs 24%, P = .03), and more frequent progression of the disease (23.29% vs 5.56%, P = .043) were found in nonsegmental vitiligo. Hyperpigmented rims surrounding patches of vitiligo were only seen in nonsegmental vitiligo (8.99% vs 0% (P = .007). Sixty-four children (56%) had laboratory investigations performed; thyroid abnormalities were found only in nonsegmental vitiligo (11.23% vs 0%, P = .0001). LIMITATIONS: Not all patients underwent laboratory investigations. CONCLUSIONS: Segmental and nonsegmental types of vitiligo have distinguishing clinical characteristics.

Brain magnetic resonance imaging in patients with Cowden syndrome.

Cowden syndrome (CS) is a rare autosomal dominant genodermatosis, characterized by multiple hamartomas, particularly of the skin, associated with high frequencies of breast, thyroid, and genitourinary malignancies. Although Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the cerebellum, a slowly progressive unilateral tumor, is a major criterion of CS, its frequency in patients with CS is unknown. Other cerebral abnormalities, especially meningioma and vascular malformations, have also been described, albeit rarely, in these patients. The aim of the current study was to use cerebral magnetic resonance imaging (MRI) to evaluate LDD frequency and to investigate other brain abnormalities in CS patients recruited by dermatologists. A multicenter study was conducted in 8 hospital dermatology departments between January 2000 and December 2003. Twenty patients with CS were included; specific cerebral MRI abnormalities were found in 35% (7/20) of them. Cerebral MRI revealed LDD in 3 patients, a meningioma in 1, and numerous vascular malformations in 6 patients. Five patients had venous angiomas (3 associated with LDD) and 2 patients had cavernous angiomas (1 associated with LDD and a venous angioma). The discovery of asymptomatic LDD in 3 patients and a cavernous angioma in another prompted us to perform neurologic examinations regularly and MRI to estimate the size and the extension of the tumor, and to assess the need for surgery. CS similarities with Bannayan-Riley-Ruvalcaba (BRR) are discussed because some patients could also have the BRR phenotype (for example, genital lentigines, macrocephaly, multiple lipomas) and because BRR seems to have more central nervous system vascular anomalies. Because CS signs can involve numerous systems, all physicians who might encounter this disease should be aware of its neurologic manifestations. Our findings confirm the contribution of brain MRI to detecting asymptomatic LDD, vascular malformations, and meningiomas in patients with CS.

Refractory venous leg ulcers: a study of risk factors.

BACKGROUND: Although certain risk factors for poor healing of leg ulcers have been identified, data concerning the characteristics of refractory ulcers have not been specifically studied in the literature. OBJECTIVE: To study the characteristics of refractory venous leg ulcers. METHODS: We retrospectively studied prognostic factors for healing in patients with refractory venous leg ulcers followed and treated in our dermatology department between January 1993 and January 2000. Each patient included in this study was compared with two patients matched for age and gender and presenting leg ulcers with normal healing, followed during the same period. RESULTS: Thirty-two of 571 patients with leg ulcers were included. The study population consisted of 20 females and 12 males with a mean age 73.5 years. The control population comprised 64 patients, 40 females and 24 males, with a mean age of 73 years. Univariate analysis demonstrated the negative prognostic impact of several previously identified factors (including surface area and history of the ulcer). In particular, multivariate analysis identified four main risk factors for refractory ulcer that are often associated in these patients: associated arterial disease, presence of post-thrombotic popliteal sequelae, recurrence of the ulcer, and disability. CONCLUSION: Four main risk factors that are often associated were identified, indicating the multifactorial nature of these refractory ulcers.