[Neonatal haemangiomatosis in identical twins with twin-twin transfusion syndrome]. / Hémangiomatose néonatale chez des jumeaux ayant un syndrome transfuseur-transfusé.

INTRODUCTION: Benign natal haemangiomatosis is characterised by the presence of multiple congenital haemangiomas restricted to the skin. It is differentiated from diffuse neonatal haemangiomatosis in which there is both cutaneous and visceral involvement, with higher morbidity and mortality. PATIENTS AND METHODS: Two identical twins, I and II (monochorionic placenta, biamniotic), born prematurely at 30 weeks' amenorrhoea, presented twin-transfusion syndrome resulting in retarded intrauterine growth in twin I, the donor, and incipient anasarca in twin II, the recipient. Twin I weighed 960 g while twin II weighed 1 200 g. At birth, miliary haemangiomatosis was observed in both infants (16 haemangiomas in I, 19 in II). Abdominal ultrasound and whole-body MRI performed in the two children revealed multiple angiomatous hepatic nodular lesions in I. Subsequent routine clinical and ultrasound monitoring (hepatic and cardiac) showed increased size of the haemangiomatous lesions over the first 4 months followed by stabilisation and gradual regression. No systemic therapy was required. In twin I an episode of ulceration of a neck haemangioma occurred at 5 months and a favourable outcome was obtained on administration of topical hydrocolloid therapy. DISCUSSION: Twin-transfusion syndrome affects 15 to 30% of monochorionic biamniotic pregnancies. It is a serious complication of twin pregnancies resulting from a dynamic process of interfoetal blood transfusion as a result of venous-venous or arteriovenous vascular anastomoses. In the present case, which appears to be the first reported case, it seems that these monochorionic twins, who shared the same placenta, presented haemangiomatosis simultaneously in utero, if we accept the hypothesis of grafting of emboli of placental microvessels in the formation of congenital haemangiomas.

[Chronic meningococcemia]. / Méningococcémie chronique.

INTRODUCTION: Chronic meningococcemia is a rare clinical form of invasive Neisseria meningitidis infection. We report 2 cases. OBSERVATIONS: A 39 year-old man and a 42 year-old woman had developed a widespread, fleeting and painful maculopapular cutaneous eruption over the past few weeks, associated with intermittent fever and inflammatory arthralgia. In both cases blood cultures isolated a serogroup B meningococcus that confirmed the diagnosis. Cutaneous histology revealed a non-specific image of leukocytoclastic vasculitis. Treatment with beta lactamin antibiotics was successful after respectively 3 weeks and 12 days. DISCUSSION: This septicemia is characterized by the clinical triad of cutaneous eruption, fever and arthralgia. It must not be mistaken for connectivitis because inappropriate corticosteroid prescription may provoke severe complications. Confirmation of the diagnosis is provided by the blood cultures, which should be repeated. In the case of strong clinical suspicion, the rapid improvement with antibiotics confirms the diagnosis.

A new sclerosing agent in the treatment of venous malformations. Study on 23 cases.

SUMMARY: Absolute ethanol is the most effective agent in the treatment of venous malformation (VM) although it is quite risky to use because of the danger of diffusion beyond the target. To reduce this risk, we have developed an alcoholic sclerosing solution that is less diffusible. The viscosity of absolute ethanol was enhanced with monographic ethyl-cellulose at a concentration of 5.88% ie 0.75 g in 15 ml of absolute ethanol 95%. 23 patients with VM located on the buttock (1), hand (2), leg (1) and face (19) were treated. A mean volume of 1.99 ml of the solution was injected directly into the VM. Each patient had an average of 2.8 procedures. Sixteen patients were done under general anaesthesia and seven with local anaesthesia. Evaluation was performed by the patient, the dermatologist of the treating multidisciplinary team and a dermatological group not involved in the treatment of the patients. Patients were evaluated after a mean delay of 24.52 months. Evaluation of the cosmetic result was made with a five point scale and the global result with a three point scale. VM pain was evaluated by the patients with a Visual Analogue Scale. The aesthetic results were graded as satisfactory (> 3) for the patient and the dermatologist of the multidisciplinary team. However the results were not as good with the independent dermatological group evaluation. The pain was significantly less important after the treatment (p << 0.001). Among the 23 patients, the local adverse events were nine necrosis with or without ethylcellulose fistula followed by only two surgical procedures. There were no systemic adverse events. Sclerotherapy of VM is usually performed with absolute ethanol or ethibloc. The main advantage of our sclerosing mixture is that it expands like a balloon when injected slowly in a aqueous media. Because of the important increase in viscosity the volume of injected solution is much lower than ethanol alone and the risk of systemic reactions is lower. Contrary to ethibloc, post-sclerosing surgery is not necessary because sub-cutaneous ethylcellulose disappears secondarily.

[Atrophic involution of juvenile xanthogranulomas]. / Involution atrophique de xanthogranulomes juvéniles.

INTRODUCTION: We report an unusual atrophic involution of juvenile xanthogranulomas. CASE REPORT: A newborn boy presented with 5 papular, nodular and necrotic lesions located on the upper part of the body. The diameter of the lesions ranged between 1 and 3 cm. Light microscopy showed an infiltrate with foamy and Touton cells. Langerhans' cell histiocytosis was eliminated because none of these cells showed reactivity for S100 protein and CD1a. At the age of 8 years, all the lesions had spontaneously regressed leaving unusual atrophic scars that had the same size as the active lesions. DISCUSSION: We compared the clinical, histological and evolution data of our patient with 251 published cases. The most significant clinical feature of juvenile xanthogranuloma is the spontaneous involution without any trace. However, hyperpigmentation, anetoderma or atrophy may occur. Atrophy is not frequent and can result from 2 mechanisms. Inflammation of the hypodermic tissue, which becomes atrophic and atrophy that may also result from collagen remodeling anomalies during the scarring process.