Cross species transmission of ovine Johnes Disease - Phase 1 : National Ovine Johne’s Disease Control and Evaluation Program.

Johne’s disease was investigated in fibre goats on several farms. The disease was caused by sheep [S] strains of Mycobacterium avium subsp. paratuberculosis. The infection appeared to be less severe than the same infection in sheep in that fewer goats than sheep became infected, and fewer goats than sheep developed obvious signs of the infection. However, infected goats shed the organism in their faeces and therefore were able to spread the infection to other goats and sheep. Therefore inclusion of goats in the control program for ovine Johne’s disease is justified. A communication program is recommended to advise producers that ovine Johne’s disease in goats may not be obvious and that testing should be undertaken to ensure disease is not present. The impact of ovine Johne’s disease on the fibre goat industry is projected not to be great due to the small number of herds likely to be infected

Extensive Post-zygotic Mosaicism of KRT1 or KRT10 Mutation Mimicking Classical Epider­molytic Ichthyosis

International audienceEpidermolytic ichthyosis (EI) is a rare disorder of keratinization belonging to the group of keratinopathic ichthyosis. EI is an autosomal dominant disease due to mutations in the genes encoding keratin 1 (KRT1) or keratin 10 (KRT10) expressed in the suprabasal layers of the epidermis (1). EI is characterized by erythroderma, blistering and erosions at birth, followed by generalized hyperkeratotic and verrucous lesions from early childhood. The lesions are generalized and palmo-plantar keratoderma may be seen (2). EI is a severe disease due to skin aspect, itching and recurrent episodes of skin infections with malodorous skin. Histological examination of skin lesions shows thickening of the stratum corneum, vacuolar degeneration of the epidermal suprabasal layer, and some clumping of tonofilaments in the keratin. Whereas EI is in the form of generalized skin lesions, localized segmental lesions along the Blaschko’s lines have been reported rarely as a consequence of post-zygotic somatic mutations in KRT1 or KRT10. Post-zygotic mosaicism in EI must be distinguished from epidermolytic naevi, a naevoid variant of keratinopathic ichthyosis characterized by localized hyperkeratotic lesions present at birth without an initial phase of blistering (3, 4). Post-zygotic mosaicism in EI usually has a limited distribution. We report here 2 cases of EI with an extensive distribution

Le bébé collodion

International audienceLe terme de bébé collodion (BC) a été introduit en 1884 par Hallopeau pour décrire un nouveau-né présentant une membrane parcheminée recouvrant l’ensemble de son tégument [1]. Le phénotype BC est rare, estimé entre 1 pour 50 000 et 1 pour 100 000 naissances. Les séries de la littérature sont rares et rapportent de manière rétrospective au maximum une trentaine de cas. Il s’agit d’un état néonatal transitoire mais qui peut engager le pronostic vital à court terme en raison des complications. La mortalité est difficile à chiffrer précisément et a diminué au cours des années avec les avancées de la néonatologie. Les chiffres selon les publications varient entre 5 %, et 24 %. Le pronostic à long terme dépend de la maladie de la kératinisation sous-jacente, le plus souvent sévère, mais des issues favorables existent