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Ehlers–Danlos syndrome type VIII: biochemical, stereological and immunocytochemical studies on dermis from a child with clinical signs of Ehlers–Danlos syndrome and a family history of premature loss of permanent teeth
Author(s) -
DYNE K.M.,
VITELLAROZUCCARELLO L.,
BACCHELLA L.,
LANZI G.,
CETTA G.
Publication year - 1993
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1993.tb00211.x
Subject(s) - ehlers–danlos syndrome , dermis , procollagen peptidase , connective tissue , pathology , type i collagen , connective tissue disease , collagen, type i, alpha 1 , medicine , anatomy , biology , endocrinology , extracellular matrix , microbiology and biotechnology , autoimmune disease , disease
Summary We studied collagen expressed by skin fibroblasts in culture, and performed immunocytochemical, ultrastructural and stereological analysis of dermis from a child with signs reminiscent of a mild Ehlers–Danlos syndrome (EDS) type IV and severe periodontitis. No alterations in types I and II collagen synthesis and secretion, or serum levels of type III procollagen aminoterminal propeptide were found, and morphological studies revealed non‐specific alterations of collagen and elastic components observed in many connective tissue disorders.