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Biomedical subjects

M Ledoux-Corbusier

Publications and source records attributed to M Ledoux-Corbusier.

4 recordsLinked to original sources

Pinguecula and actinic elastosis. An ultrastructural study.

Three cases of pinguecula (conjunctival elastosis) were ultrastructurally investigated. Findings were compared with the features described in cutaneous actinic elastosis. Changes observed were not uniform. In both diseases, numerous elastotoc fibers were present with a finely granular matrix and masses of dense grains. The degenerative changes of the elastotic fibers evolved differently in conjunctival stroma and in dermis. The superficial extracellular concretions observed in pinguecula seemed to be an ultimate stage of elastotic degeneration. Like actinic elastosis and elastosis observed in chronic radiodermatitis, pinguecula is believed to result from a dystrophic increased elastogenesis induced by chronic irradiation, with secondary degenerative changes.

Aged

[Complete alpha-1-antitrypsin deficiency in a patient with Ehlers-Danlos syndrome].

Complete alpha-1-antitrypsin deficiency of the type PiOO associated with chronic obstructive lung disease, cutaneous hyperextensibility and hyperlaxity of joints were found in a nineteen-year-old Moroccan boy. On a nosological point of view, this patient could be included as a 8th form, in the Ehlers-Danlos syndrome which now groups seven clinical variants. A causal relationship between the biological deficiency and the clinical alterations (pulmonary, cutaneous and articular) could be assumed according to the biological and ultrastructural findings.

Adult

alpha1-Antitrypsin deficiency and skin abnormalities.

A 19-year-old Moroccan male was found to have total absence of serum alpha1-antitrypsin, a major inhibitor of elastase. This patient had chronic obstructive lung disease, hyperextensibility of the skin over the cheeks and wrists, and hyperlaxity of the hand joints. Microscopic sections of the skin revealed a thickened dermis with shortened and rarefied elastic fibers. Ultrastructural study showed collagen fibers with variable and irregular diameters. Elastic fibers were scarce and their relatively poor matrix was surrounded by numberous microfibrils. The outline of the fibers was irregular with deep recesses filled with microfibrils. The ergastoplasm of the fibroblasts was well developed. The differential diagnosis with other connective dystrophies showed the original characteristic of this case. Clinically and histopathologically, the skin abnormalities are probably related to the deficiency in elastase inhibitor.

Adult