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Biomedical subjects

M Cony

Publications and source records attributed to M Cony.

8 recordsLinked to original sources

[Langerhans-cell histiocytosis and Erdheim-Chester disease: probably not a fortuitous association].

BACKGROUND: Erdheim Chester disease (MEC) is a rare non-Langerhans cell histiocytosis characterized by multi-visceral involvement. We report a case of MEC associated with Langerhans cell histiocytosis (HCL). CASE REPORT: A 46-year-old women presented skin and vulvar localization of HCL associated with typical MEC bone involvement. Despite chemotherapy (vinblastine) and prednisone, the disease progressed to involve the central nervous system, leading to fatal outcome. Post-mortem examination showed HCL in skin, MEC in bones and central nervous system, and intermediate histiocytic proliferation in the encephalon. DISCUSSION: Usually, MEC and HCL are considered as distinct entities. MEC is characterized by a xanthogranulomatous proliferation of CD 68+ foamy histiocytes nested in fibrosis, and HCL by a proliferation of PS 100+ and CD1a+ Langerhans cells. However, our observation, as well as previous reports, suggests that MEC is part of the HCL spectrum.

Bone Diseases↗

Treatment of port-wine stains with the 585-nm flashlamp-pulsed tunable dye laser: a study of 74 patients.

Over 20 months, we have treated 74 patients (59 children less than 12 years of age) with port-wine stains (PWS) using a 585-nm flashlamp-pulsed dye laser (SPTL-1, Candela Corp., Wayland, Mass., USA) after topical anesthesia with Emla cream. A 5-point reference color scale was used to evaluate the results. 45 patients had at least one treatment on the entire surface of the lesion. A mean of 88 impacts per session was delivered. There was a significant decrease in color in around two thirds of the cases after one complete treatment with a gradual tendency to improvement after subsequent treatments. Younger age at the beginning of treatment was not found to be predictive of a better outcome after the first treatment. In around one third of the cases, positive test site treatment was not correlated with significant improvement after one treatment. Lesions situated on the limbs were less responsive than those on the head and neck. Except for problems due to absence of general anesthesia in young children enduring repeated stressful and sometimes painful procedures, the overall impression is that early treatment of PWS is possible with very limited risks of scarring using this technique.

Adolescent↗

[Periungual capillaroscopy patterns in normal children].

In children as well as in adults, capillaroscopy is an unsophisticated and non invasive technique which allows to investigate vascular acrosyndromes and systemic diseases. We have studied nailfold capillaroscopy patterns in 80 children without over vascular or systemic disease: pericapillary halos and haemorrhages increased with age and capillaries matured towards the typical hair pin structure seen in adults. The number of minor dystrophies increased with age and the venous subpapillary plexuses became less easily visible.

Adolescent↗

[Lichenoid pityriasis (parapsoriasis guttata) in children. Report of 17 cases].

Seventeen cases of pityriasis lichenoides diagnosed over a nine-year period in children under 15 years of age are reported. Patients with this benign disease develop papular skin lesions covered with thick, coherent scales which detach in a single piece (reminiscent of sealing wax). Pruritus is not marked. Lesions may be necrotic (Mucha Habermann's small pox-like form, n = 6) or mild (leukodermic form, n = 2). Half of the patients studied developed several episodes and total duration of the disease exceeded two years in one third of cases. Recovery occurred after one or two episodes in half the children. Scars developed in some patients with severely necrotic lesions. None of the patients developed lymphoma. All patients with lymphomatoid papulosis progressing to lymphoma reported in the literature were adults. Pathogenesis of pityriasis lichenoides remains unknown but may involve lymphocytic vasculitis. No truly effective therapy is available. However, oral macrolides can be used especially in patients with early manifestations suggesting an infectious disease. Emollients, heliotherapy and ultraviolet therapy may also be recommended.

Adolescent↗

Treatment of leg ulcers with an allogeneic cultured-keratinocyte-collagen dressing.

A living cellular allogeneic dressing made up of cultured keratinocytes adhering to a collagen film was used to treat 20 leg ulcers of various aetiologies in 16 patients. A reduction in pain was noted in 80% of cases, and promotion of granulation tissue in the ulcer bed in 70% of cases. In 10 patients, epithelialization of 71 +/- 29% of the ulcer was noted at Day 30.

Aged↗