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J Fernandez-Diez

Publications and source records attributed to J Fernandez-Diez.

3 recordsLinked to original sources

Tamale foot: deposit of acid mucopolysaccharides in the synovial sheaths of extensor tendons of the foot, resembling tendinitis, in a patient with juvenile ankylosing spondylitis.

We describe a clinical case of juvenile ankylosing spondylitis (AS) that developed "pseudo-chronic tendinitis" of the foot. A 20-year-old male patient had HLA-B27 positive juvenile AS since he was 13 years old. At the age of 19 he presented chronic pain in the dorsum of the left foot. Examination disclosed an increased volume of the tarsal dorsum, with rubbery consistency, with no evidence of venous or lymphatic insufficiency, godette, or inflammation in laboratory tests, giving the foot the appearance of a tamale. Synovectomy of the foot extensor tendon sheath was followed by relief of pain and swelling. Histopathological study showed a deposit of acid mucopolysaccharides (MPS) with no inflammatory cell infiltrate. Tamale foot in juvenile AS may develop as a consequence of acid MPS deposit with no evidence of synovial inflammation. The good response to synovectomy suggests this is the preferred treatment for tamale foot.

Adolescent↗

Vascular changes in cutaneous neurofibromas.

A variety of vascular changes has been associated with neurofibromatosis, and morphological alterations of arteries have been described previously. However, little attention has been paid to structural modifications of the microvasculature in neurofibromas themselves. Small vessels of 10 cutaneous neurofibromas, excised from patients with neurofibromatosis, were studied by using transmission electron microscopy and compared with normal skin vessels. The major alterations were: (1) increased thickness of the cytoplasm of endothelial cells which send numerous long processes to the lumen and, in some instances, obliterate it; (2) larger numbers of endothelial cell processes on the abluminal surface which are apposed to pericyte processes; (3) increased amounts of filaments and free ribosomes in the cytoplasm of endothelial cells, and (4) abundant pericytes with bundles of cytoplasmic filaments and many small subplasmalemmal densities. Mitotic figures were not seen in any of the vascular elements. These changes were interpreted to be the result of a generalized malformative process of blood vessels in neurofibromas rather than a consequence of the action of a tumor angiogenesis factor.

Adolescent↗