Diseases of the nervous system / edited by Archibald Church.

Date:
1908
Catalogue details

Licence: In copyright

Credit: Diseases of the nervous system / edited by Archibald Church. Source: Wellcome Collection.

    1184/1256 (page 1142)
    There are rarely any objective sensory disturbances, even in the parts which are most markedly altered. Trophic changes are not limited to the skin but affect also other tissues, and in the light of most recent investigations we may state that all the tis- sues of the body are affected. The changes, as a rule, pass directly from the skin to the muscles, but occasionally the muscles are affected in areas in which cutaneous changes are either slight or wholly absent. I recently observed a case in which the myoscleroses were much more extensive and intense than the dermatoscleroses. Of course this increases the difficulty of differentiating myositis interstitialis, muscular induration. Furthermore, there is atrophy of the hones with shortening and thinning (acromicria). I was several times able to determine this in the X-ray picture. If the joints are implicated, the differentiation from chronic articular rheumatism becomes very difficult. Only exceptionally are the mucous membranes affected. A significant fact in the prognosis is that the internal organs may be affected by sclerotic and atrophic processes, and cachexia often appears in the later stages of the disease; thus scleroderma finally becomes a systemic affection. The manner in which sclerodermatic changes appear has led to the sub- division of scleroderma into distinct forms. Scleroderma of the entire body is quite rare; in such extreme cases the individual resembles a mummy, a condition Grasset has described by word and picture. In contrast to this scleroderma diffusa, which is not always complete, js scleroderma circum- scripta, in which we recognize the striaform or flake-like arrangement of the sclerodermatous plaques. Under diffuse scleroderma, owing to its special localization, we differentiate sclerodactylia which chiefly affects the hands, more rarely the feet. This latter form bears, as I have several times indi- cated, the closest relation to other vasomotor trophic neuroses. It is cer- tainly not a disease sui generis, but besides its localization it has a few indi- vidual characteristics which I must mention: The frequent implication of the deep-lying tissues, the tendons, fascia, muscles, joints and bones, the well marked vasomotor and sensory disturbances which often accompany these conditions, the frequent deviation of the trophic disturbances from the true sclerodermatic type so that, besides scleroderma, more or less distributed gangrenous processes also occur. These cases are so closely related to Ray- naud’s disease that, not rarely, their positive differentiation from it is im- possible. The relations between scleroderma and hemiatrophic faciei progressiva are very close. Hutchinson simply classifies the latter as scleroderma local- ized to the region of the trigeminal nerve. It usually affects young persons, most commonly those between 10 and 20 years of age; we are uncertain as to the cause, but a neurotic predisposition appears to play a role. It begins gradually; in a limited area, perhaps in the brow or cheek, there is atrophy of the skin, as well as of the subcutaneous tissue, the bones, and also the muscles. From this starting point atrophy involves gradually the entire half of the face; there is a diminution of the size of all parts. Changes in the pigment, and anomalies in the secretion of sweat, in the secretion of the sebaceous glands, in the temperature of the skin, and in the growth of the hair appear, similar to those of scleroderma. Just as in the latter affection, there are no objective sensory disturbances, but paresthesia and even severe