A four-generation Ehlers-Danlos syndrome with vascular dissections. Skin ultrastructure and biomechanical properties.

Ehlers-Danlos syndrome (EDS) is heterogenous with regard to genetic traits, clinical manifestation, the biomechanical and microscopic properties of connective tissues, and basic molecular defects. We report on nine relatives of four generations who suffered from large vessel dissections and cutaneous microscopic changes consistent with EDS. Measurements of the mechanical properties of skin were performed using a computerized suction device (Cutometer). Morphological and biomechanical alterations suggestive of EDS were present in all examined subjects. A loose network of collagen bundles was admixed with clumsy elastic fibres. Factor XIIIa-positive dermal dendrocytes looked almost normal but were slim and rarefied in four subjects. The severity in ultrastructural alterations of the collagen network differed among the subjects. The group with the most prominent changes showed the most striking biomechanical alterations characterized by increased biologic elasticity without any excess in skin extensibility. A positive correlation was found between skin extensibility and elasticity. In conclusion, distinct alterations in the collagen scaffolding were found to be correlated to variable severity in biomechanical alterations of the skin. The predictive value of these changes for large vessel dissections in some families at risk remains to be settled.

[How I treat....A stoutness-associated intertrigo]. / Comment je traite... un intertrigo lié à l'embonpoint.

Intertrigo is a common inflammatory disorder of various origins. It can represent a single skin manifestation or be part of a disorder possibly exhibiting specific manifestations on other parts of the body. Hence, intertrigo may represent a sign of a peculiar disease. It may also result from physicochemical aggressions of the skin following occlusion of the large skinfolds particularly in case of stoutness. In this intance, a treatment similar to that of diaper dermatitis can be offered. A paste enriched or not with miconazole nitrate is indicated to prevent or treat most presentations of intertrigo.

[Electromagnetic lipolysis and semicircular lipoatrophy of the thighs]. / Lipolyse électromagnétique et lipoatrophie semi-circulaire des cuisses.

INTRODUCTION: The semicircular lipoatrophy of the thighs is a disorder whose incidence remained rare for years. Only recently, an "epidemic" situation emerged, affecting hundreds of subjects who shared a status of administrative employee. OBSERVATION: The older etiopathogenic hypotheses give way to a newer one implying the electromagnetic fields generated by computers and their wirings. The resulting modifications in the intrinsic bioelectrical properties of the skin could influence the biology of macrophages exhibiting lipophagic activity in the hypodermis. The electroactivation of these cells could lead to TNF-alpha release. DISCUSSION: The semicircular lipoatrophy of the thighs is a problem that may affect an employee out of two. Rather than hoping an efficaceous drug therapy, prevention must be advocated by adapting the work conditions related to the use of computerized devices.

[How I treat...topical treatment of plaque type psoriasis by the combination of calcipotriol and betamethasone dipropionate]. / Comment je traite ... un psoriasis en plaques par l'association calcipotriol-dipropionate de bétaméthasone.

The combination of calcipotriol and betamethasone dipropionate in a single topical formulation is a recent advance in treatment of plaque type psoriasis. Studies rooted on evidence-based medicine have been recently published. We report a synthesis and a critical analysis, critical analysis of these investigations, and translate their data with a view to apply them in daily practice.

Cutaneous myospherulosis and membranous lipodystrophy: extensive presentation in a patient with severe steroid-induced dermal atrophy.

Myospherulosis is a rare disorder that may develop in various organs including the skin. It is characterized by a typical histological presentation resulting from the interaction between erythrocytes and lipids. We present a case of cutaneous myospherulosis associated with membranous lipodystrophy. The lesions were supervening on severe steroid-induced skin atrophy upon which ointments had been applied.

[Cutaneous stigmata of diabetes mellitus]. / Stigmates cutanés du diabète.

A number of relationships exist between diabetes mellitus and a series of cuteanous disorders. Skin lesions may reveal diabetes in a more or less specific way. They may also represent complications supervening in an already treated diabetic patient. Some of these dermatoses (acanthosis nigricans, purpuric and pigmented capillaritis) are markers of macrovascular complications. The same disorders and some others (xerosis, Dupuytren's disease) are more frequently associated with microangiopathy in Type II diabetes. Other skin diseases (alopecia areata, vitiligo) are markers of auto-immunity in Type I diabetes.

[Skin and its ethnic shades]. / La peau et ses nuances ethniques.

Ethnic (racial) differences exist for a series of physiological skin characteristics. Colour is responsible for typology and resistance to ultraviolet irradiations. Other differences have also been reported for the transepidermal water loss, the size and cohesion of corneocytes, the vascular reactiviy, the biomechanical properties, the pH, the lipidic content, the superficial microflora and the structure of mast cell granules.

New insights into the effect of amorolfine nail lacquer.

Despite improvements in antifungal strategies, the outcome of treating onychomycoses often remains uncertain. Several factors account for treatment failure, of which the pharmacokinetics and pharmacodynamics of the antifungal are of importance. The taxonomic nature and ungual location of the fungus cannot be neglected, besides the type of nail and its growth rate. In addition, the biological cycle of the fungus and the metabolic activity of the pathogen likely play a marked influence in drug response. The presence of natural antimicrobial peptides in the nail is also probably a key feature controlling the cure rates. There are many outstanding publications that cover the full spectrum of the field. The purpose of this review is to put in perspective some facets of activity of the topical treatment using amorolfine nail laquer. The antifungal activity of the drug is likely less pronounced in onychomycosis than that expected from conventional in vitro studies. However, the nail laquer formulation should reduce the propensity to form antifungal-resistant spores and limit the risk of reinfection.

[The calcipotriol-betamethasone dipropionate association]. / Le medicament du mois. L'association calcipotriol-dipropionate de bétaméthasone (dovobet).

The topical treatment of psoriasis benefits from the alternate use of dermocorticosteroids and vitamin D3 analogues. A new galenic formulation allows to combine them in a single application. Dovobet (LEO Pharma) ointment is the association of calcipotriol 50 microg/g with betamethasone dipoprionate 0.5 mg/g. This formulation boosts the therapeutic activity of calcipotriol. It also decreases the irritative inflammatory reaction due to calcipotriol without increasing the atrophogenic risk of the dermocorticoid.

[How I treat ...scalp psoriasis]. / Comment je traite...un psoriasis du cuir chevelu.

Scalp involvement by psoriasis requires a ted treatment suited to the particularities of the anatomical site In course of time, the improvement of therapeutic agents and the galenic refinement have enhanced both the efficacy and cosmetic acceptability of the scalp care. The main therapeutic classes encompass keratolytics, dermocorticosteroids and vitamine D3 analogues. The patient compliance is reported to be best for the liquid or foam formulations.

[Syphilis: a STD back to us]. / Le cas clinique du mois. La syphilis, une MST de retour en nos murs.

Sexually transmitted diseases (STD) show frequent epidemiological changes. We report a case of secondary syphilis diagnosed in a 32-year old woman during post-partum. The disease, although showing a typical presentation, raises the problem of dating with precision the contamination time. This is of importance for treating the newborn at risk of congenital syphilis. We highlight the constant attention that should be brought to disclose this venereal disease that shows so variable clinical and histological presentations. Syphilis seems on the rise in the Mosan valley and in some cities where prostitution is imported from Eastern Europe and Africa.

[Severe paroxysmal drug eruptions]. / Les toxidermies paroxystiques graves.

Some drug reactions showing cutaneous expression exhibit a paroxysmal course. These diseases encompass the toxic epidermal necrolysis, the drug hypersensitivity syndrome, and the acute generalized exanthematic pustulosis. These syndromes are associated with dismal outcome. They represent medical emergencies needing hospitalization in specialized care units.

[Paroxysmal acne]. / Acnés paroxystiques.

Contrasting with the chronic evolution of acne vulgar, other types of acne are characterized by paroxysmal outbreaks. They can be endogenous or exogenous in origin. Each of these two groups can be further divided into separate entities.

[How I explore ... diaper dermatitis]. / Comment j'explore ... les dermites des langes.

Diaper dermatitis is a frequent condition. Several clinical types are distinguished. The most frequent type results from increased fragility of the newborn buttock skin when covered by diapers. According to the mechanisms involved and the severity of the dermatitis, one can distinguish the intertrigo of the chubby baby, and the so-called "W", "Y" and "red panties" types of diaper dermatitis. When the effects of occlusion are not controlled by adequate absorption by the diapers, hyperhydration of the stratum corneum occurs and progresses to maceration. As a result, the value of the coefficient of friction of the skin increases with epidermal weakening to rubbing. In addition, fecal enzymes (urease, proteases and lipases) alter urines and skin. Judicious hygiene measures and the correct choice of diapers are mandatory. Cutaneous colonization by microorganisms, the most important of which being Candida albicans, is the main complication. Adequate preventive and curative measures can combat diaper dermatitis with confidence. A miconazole paste allows to improve the tribological properties of the interface between diapers and the skin. It also abates the impact of Candida albicans in the pathogenesis of the skin disorder.

Translocation (16;17)(q22;p13) is a recurrent anomaly of aneurysmal bone cysts.

Recently, Panoutsakopoulos et al. (1999) reported 2 cases of aneurysmal bone cysts with a recurrent (16;17)(q22;p13) translocation. We present here two additional cases harboring the same translocation as well as additional chromosomal changes.