title: [Acrogeria. Clinical, histopathologic and ultrastructural study].
abstract: 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.
mesh_terms: D002648:Child; D005260:Female; D005533:Foot Dermatoses / Q000151:congenital* / Q000473:pathology; D006229:Hand Dermatoses / Q000151:congenital* / Q000473:pathology; D006801:Humans; D008297:Male; D012867:Skin / Q000473:pathology / Q000648:ultrastructure; D012871:Skin Diseases / Q000151:congenital* / Q000473:pathology
vernacular_title: L'acrogeria. Etude clinique, histopathologique et ultrastructurale