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[Costa's acrokerato-elastoidosis. Ultrastructural study (author's transl)]

R Massé, A Quillard, B Héry

    Annales De Dermatologie Et De Venereologie
    |June 1, 1977
    PubMed
    Summary

    Acrokerato-elastoidosis presents with skin lesions on hands and feet. Ultrastructural analysis reveals abnormal elastic tissue and fibroblast issues, suggesting problems with elastic material processing.

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    Area of Science:

    • Dermatology
    • Histopathology
    • Biochemistry

    Background:

    • Acrokerato-elastoidosis (AKE) is a rare genetic skin disorder.
    • It is characterized by distinctive keratotic papules and translucent papules on the dorsal hands and feet.

    Observation:

    • Ultrastructural examination of AKE lesions was performed.
    • The study focused on the morphology of elastic tissue and fibroblasts within the affected skin.

    Findings:

    • The elastic tissue in AKE lesions was found to be rarefied and abnormally structured.
    • Fibroblasts exhibited peripheral dense granules and a lack of extracellular elastic fibers.

    Implications:

    • These findings suggest a potential defect in the synthesis or secretion of elastic material by fibroblasts in AKE.

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  • Understanding these ultrastructural alterations may provide insights into the pathogenesis of AKE and related connective tissue disorders.