Abstract
A case of Gottron’s syndrome or symmetrical progressive erythrokeratoderma is presented. Typical, symmetrical erythematosquamous lesions are limited to the limbs and face; the disease appeared at 4 months and stabilized at 3 years. The histological study discovers only nonspecific granular layer alterations.The ultrastructural investigation reveals an important intercellular edema and a moderate cytoplasmic vacuolization in the basal and prickle cell layer and in the stratum corneum; wandering lymphocytes are also observed. The association with neuropsychological and sensorial troubles as well as facial dysmorphy and kidney abnormalities is underlined.
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© 1989 S. Karger AG, Basel
1989
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