A cutaneous disorder of connective tissue in amyotrophic lateral sclerosis

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Abstract
Deltoid or abdominal biopsy specimens were obtained from 46 cases of amyotrophic lateral sclerosis (ALS), 58 patients with other diseases and 15 healthy persons. On the basis of histochemical staining the biopsies were classified as "diagnostic of ALS", "not ALS" or "questionable". A diagnosis of ALS was based upon demonstration of an elastosis, an increased amount of mucopolysaccharide through the dermis, degeneration of the arrector pili muscles, focal areas of altered connective tissue and altered collagen. The last 2 mentioned changes are considered the most characteristic. There was little correlation between age and histologic classification and the origin of the patients had no influence on the findings. Preliminary data suggest that the changes are not secondary to local nerve or cord lesions or related to cutaneous readjustments to diminishing muscle mass. The findings indicate the possibility of defective carbohydrate metabolism of the connective tissue and epidermal cells. The accuracy of diagnosis seems to be influenced by the severity of the disease.

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