[Ectodermal dysplasia with anhidrosis (a recent case)].
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Biomedical subjects
Publications and source records attributed to P Agache.
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About a recent case of acrogeria a review of the 18 cases of the litterature defines the clinical and evolutive characteristics of this primary atrophy of the skin. The histopathological and ultrastructural study of the pathological dermis with regard to a reference specimen of same age and sex shows fibroblastic and fibrillary alterations. The hyperplastic orceinophilic material is mainly of the "pseudoelastic" type. The collagenous fibers are hypoplastic and often immature. An abundant granulofilamentous substance is visible in the extracellular space and in the fibroblasts proper; it is often intricated with the pseudoelastine and contains immature collagenous fibers. The cytoplasm of the fibroblasts is swollen by ergastoplasmic vacuoles filled with the granulofilamentous substance and contains numerous pseudomyelinic bodies. All these anomalies suggest that acrogeria could be a disease of the fibroblast.
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This paper describes a new case of Incontinentia Pigmenti in a newborn. This genodermatosis develops mostly in females and three clinical stages are recognized. Other ectodermal abnormalities may be associated with a risk for the prognosis. The clinical, histological features and differential diagnosis are discussed.
Our observation concerns a patient aged 29 who has presented for the last two years a smooth lesion, non-infiltrated, golden yellow, and situated on the interior surface of the left knee. This lesion recalls the lichen aureus confirmed by standard histology nad Perls' coloration. There is capillary fragility without plaque anomaly. There is, however, no sign of subjacent venous incompetence.
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