Louis-Bar syndrome; Boder-Sedgwick syndrome


Autosomal, recessive, multisystem disorder characterized by progressive neurological impairment, cerebellar ataxia, variable immunodeficiency, impaired organ maturation, x-ray hypersensitivity, ocular and cutaneous telangiectasia, and a predisposition to malignancy


Unclear; possibly associated with dysregu-lation of the immunoglobulin gene super-family, which includes genes for T-cell receptors; abnormal sensitivity to x-rays and certain radiomimetic chemicals, possibly leading to chromosomal abnormalities, infections, and malignancies

Clinical manifestation

Ocular and cutaneous telangiectasia; neurological abnormalities, mainly ataxia, abnormal eye movements, and chore-oathetosis

Differential diagnosis

Telangiectatic diseases: hereditary hemor-rhagic telangiectasia; chronic liver disease; benign essential telangiectasia; sun damage; neurologic disorders; Friedreich disease; cerebral palsy; familial spinocerebel-lar atrophies; GM1 and GM2 gangliosi-doses; progressive rubella panencephalitis; subacute sclerosing panencephalitis; postinfectious encephalomyelitis; cerebel-lar tumor


No effective therapy


Gatti RA (1995) Ataxia-telangiectasia. Dermato-logic Clinics 13(1):1-6

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

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