Resolution and repair of elastic tissue calcification in pseudoxanthoma elasticum
Martinez-Hernandez, A.; Huffer, W.E.; Neldner, K.; Gordon, S.; Reeve, E.B.
Archives of Pathology and Laboratory Medicine 102(6): 303-305
1978
ISSN/ISBN: 0003-9985 PMID: 580723 Document Number: 137352
Biopsy specimens of a skin lesion from a patient with pseudoxanthoma elasticum [an inherited connective tissue disorder with dermal, vascular and ocular involvement] were obtained at the beginning and end of a 1 1/2 yr period of clinically documented regression of the lesion. Light microscopic studies of these specimens showed that, during the period of regression, there was a decrease in the number of abnormal calcified elastic fibers in the dermis. EM findings were interpreted as indicating breakdown and removal of mineralized elastic fibers at the beginning and formation of new elastic fibers at the end of the regression period. Cytoplasmic processes of active fibroblasts were intimately associated with fibers undergoing dissolution. The case thus represents a clinically and morphologically documented example of resolution and repair of elastic tissue calcification.