A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon.

A vascular mass localized in the face and the neck was displayed by ultrasonography in a 38-week-old male fetus. At birth, the mass was bulky and purplish. The newborn breathed spontaneously but with severe desaturation. During laryngoscopy, we observed an obstruction of the larynx with a left-shift caused by the hemorrhagic mass. Blood analysis revealed anemia, severe thrombocytopenia, and coagulation disorders. The diagnosis of kaposiform hemangioendothelioma (KHE) complicated by a Kasabach-Merritt phenomenon (KMP) was put forward and treatment with propranolol, corticoids, and vincristine was initiated. Platelets were transfused daily for 8 days but did not resolve the thrombocytopenia. At day 8, we added sirolimus to the treatment and noted a rapid response with the normalization of the platelet count within 1 week and a significant regression of the mass. In this paper, we review the clinical and biological features of hemangioendothelioma associated with KMP and discuss its current and future treatment. Sirolimus seems to be very promising.

[Dermatological particularities and pathologies of premature infants]. / Particularités et pathologies dermatologiques du nourrisson prématuré.

In contrast with the full-term infant, the skin of the preterm neonate is structurally and functionally immature, especially birth occurred before 30 weeks gestation. The inefficiency of the epidermal barrier may result in dehydration, thermal instability and toxic reactions from percutaneous absorption of topically applied agents. An increased risk for bacteremia and sepsis exist because of the easily injured skin, combined with compromised immunity. The present article summarizes the consequences of this skin immaturity and the different means to avoid them. We shall also describe 2 pathologies more frequent in premature infants: sclerema neonatorum and acquired zinc deficiency.

Squamometry in seborrheic dermatitis.

BACKGROUND: Seborrheic dermatitis is a corticosteroid-responsive condition. Topical corticosteroids exhibit distinct activities according to the type and cellular site of action of the hormone and its vehicle. The latter influences not only drug penetration, but also the structure and function of the horny layer. OBJECTIVE: The present study compared the early response of seborrheic dermatitis to two topical corticosteroids using objective quantifications of the stratum corneum alterations. METHOD: Squamometry was used to supplement the clinical assessment of the therapeutic activity of 0.1% hydrocortisone butyrate in an oil-in-water emulsion (Locoid Crelo) and 0.05% betamethasone 17-21 dipropionate lotion (Diprosone lotion). RESULTS: The hydrocortisone butyrate formulation had a faster onset of efficacy as documented by a greater improvement of lesions after a 1-week twice-daily treatment. Clearance or marked improvement was observed in the two treatment groups at completion of the 2-week trial. CONCLUSIONS: As the ranking of the clinical efficacy was not predicted by the intrinsic anti-inflammatory potency of the steroids, it is suggested that the vehicle contributed itself to the global in vivo efficacy. The anti-inflammatory action of hydrocortisone butyrate appears to be synergistically implemented by the emollient effect of the specially designed oil-in-water emulsion.

[Cutaneous melanomas, a spectrum of emerging cancers in women of Wallonia. Outlook by the Mosan Study Group of Pigmented Neoplasms]. / Les mélanomes cutanés, un spectre de cancers émergents chez la femme en région Wallonne. Un regard porté par le Groupe Mosan d'Etude des Tumeurs Pigmentaires.

The Mosan Study Group of Pigmented Neoplasms was founded about 15 years ago. It has collected more than 20,000 cutaneous malignancies including melanomas and basal and squamous cell carcinomas. The incidence of these cancers is on the rise in Wallonia. In particular, malignant melanomas represent a spectrum of emerging cancers characterized by a proteiform biological outcome. They mostly affect young women. The major risk factor appears to be iterative and unwise ultraviolet exposures. The prevention of melanomas is basically founded on such a dogma and accordingly relies on sunscreens. However, controversies about their beneficial effects are rife and fueled by axiomas and contradictory sophisms. At the exception of surgery, the therapeutic options for the diverse types of melanomas do not yet fulfill the scope of evidence-based medicine.

[Atopic dermatitis]. / La dermatite atopique.

For the past decades, the prevalence of atopic dermatitis is on the rise. Three phases of the disease are recognized according to the age of the patient, either in the newborn age, in infancy or after puberty. A partial enzymatic defect in delta-6 desaturase has been reported. Such metabolic anomaly would be responsible for the alterations in both the skin barrier function and in the Th2 inflammatory reactions in part mediated through IgE. Some microorganisms colonizing the skin play an important causal role in the clinical exacerbations. The influence of food allergy on atopic dermatitis remains obscure and often unconvincing. By contrast, irritant and allergic challenges from the environment exhibit an undisputable worsening effect. The prevention relies on the eviction of predisposing factors and is favourably influenced by emollients specifically designed for atopic dermatitis. A food supplementation in alpha-linoleic acid and some specific chinese herbal remedies might well be useful. The treatment relies primarily on topical applications of corticoids. Puva-therapy, UVB phototherapy and immunomodulation by cyclosporine are indicated for difficult cases.

Congenital psoriasis following the lines of Blaschko.

Congenital psoriasis without an underlying nevus following the lines of Blaschko is described in a 6-year-old girl. In addition to this special type of psoriasis, she suffered from atopic dermatitis. The lesions were aggravated by sun exposure and phototherapy treatments, as well as by upper airways infections, without any clear explanation of these trigger factors. We propose to call this form of psoriasis congenital blaschkoid psoriasis.