Symptoms. – This comparatively rare affection often follows exposure to cold and wet, with antecedent rheumatic pains and stiffness in the limbs or joints and other subjective sensations. The onset may be insidious and not clearly noted by the patient, or the skin symptoms may be first observed and may appear rapidly. The initial lesion is a non-inflammatory infiltration with or without edema. If with edema the surface may pit on hard pressure but owing to the density of the parts there is none of the doughy feeling of ordinary edema.
Fig. 109. – Diffuse symmetrical scleroderma, showing the hide-bound skin over the joints, atrophy of the nails and joint ankylosis. The surface conditions of this case have been much improved by Rhus tox. 3x and applications of mild X- raying and high-frequency currents.
Fig. 110. – Radiograph of hands shown in fig. 109, demonstrating the loss of the interarticular substance in the distal interphalangeal articulations.
More often edema is absent. After a variable period of time progressive hardness and rigidity symmetrically develop, but general scleroderma is very infrequent and even then the palms and soles are usually exempt. While it always attacks the upper segment of the body first, it may involve the lower simultaneously. The sites of preference in their order are the upper extremities, trunk, face, head and lower extremities. In certain locations it markedly interferes with normal functions. On the chest it may impede respiration and cause a disappearance of the female breast, or on the face restrict the movements of the mouth, eyelids and other motions of the facial muscles, causing a distinctive lack of expression.
While the affected skin merges imperceptibly into the sound, within an inch or two of the border of the indurated portion the skin will be found entirely normal. The process at its height presents a surface of leathery hardness and rigidity which cannot be impressed by the fingers or pinched into folds but a whitish line will be left after the finger nail is firmly drawn across the surface, the color returning slowly. The normal lines are largely obliterated but sensibility is usually unimpaired or only slightly increased or diminished. There may be pain on pressure and itching usually caused by some other coexisting eruption. The parts affected appear frozen but are without coldness. Various modifications or exceptional appearances have been noted. Dilated capillaries may appear in contrast with the abnormally pale surface. Brown or black pigmentation may be seen in lines, spots or generally diffused. Loss of pigment in variously sized or shaped patches will give a waxy, glistening surface like marble. Superficial or deep-seated nodules may appear and disappear and crusting or scaling may occur in spots. If the mucous surfaces of the tongue, gums, palate, pharynx, larynx and vagina are affected, the disease will manifest itself in spots and bands of sclerosed membrane.
The disease pursues an erratic but symmetric course, changing its situation or progressively extending with periods of aggravation or occasional amelioration lasting for years. Restoration or involution may follow when the maximum involvement is reached or treatment arrests the process. In such cases elasticity, mobility and function of the skin are wholly or partially restored. If this happy condition does not take place, atrophy begins with reduction of the parts involved to such an extent that the muscles may disappear and the atrophic skin seem attached directly to the bone beneath. This atrophic stage may cause the limbs of the adult to shrink to the size of a small child, the joints to become enlarged, and pseudoanchylosed. When the last named condition affects the hands, it is known as sclerodactylia. Ulceration or gangrene may follow slight injuries. On the face the features may be so distorted by shrinking and compression that the expression of the countenance is absolutely changed. Restoration to a healthy condition of the skin never occurs after the atrophic stage is well advanced, but even extensive scleroderma may be arrested by judicious treatment.
While the bodily health and general functions remain unaffected for a long time, nutrition gradually fails and the patient sinks under emaciation, insomnia, neuralgic or rheumatic pains, or mental depression, into a fatal marasmus or exhaustion. Frequently various complications intervene and hasten the ending. Sufferers from this disease are sensitive to atmospheric changes, especially cold and dampness. The duration of the disease is long but uncertain. Kaposi mentions a case which persisted for thirty-one years without the loss of the general health and one of my own shows a history of twenty-five years with the same present result. The disease in children is more acute and the tendency to atrophic changes less marked and persistent.
Etiology and Pathology. – While no exact causal reason is known, this disease is more prevalent in early adult life. However a variable proportion of from 10 to 20 per cent. have been noted before the fifteenth year. Two out of every three cases occur in women. Possible inferential causes are privations, exposure to cold or heat, rheumatism, erysipelas, traumatisms, thyroid and visceral diseases, and mental and nervous emotions. It would seem that the pathological process is an angiotrophoneurosis which produces muscular and connective tissue hypertrophy, with consequent obstructive compression of the arterial, venous and lymphatic vessels, compression of the glands and fat lobules and disappearance of the contents of the latter. In the later or atrophic stage all the anatomical parts of the skin and the tissues directly beneath are affected.
Diagnosis. – Sclerema neonatorum which closely resembles scleroderma begins in early infancy, months before the earliest known case of scleroderma. Besides, in this disease the skin is always cold. Xeroderma pigmentosum may be distinguished by its occurrence on the exposed parts of the body in tumor-like growths. Only the atrophic stage of this disease is like scleroderma.
Prognosis and Treatment. – At least one-half of the cases may be improved or the process arrested. Hence there is always the hope of recovery before the advent of the atrophic stage. In the extreme form of this later state the prognosis is extremely unfavorable as to recovery or long life.
Treatment is hygienic and constitutional. Tonics are often indicated and the diet should be plain, nutrition and ample. Clothing should be non-irritating and warm so as to protect against the changes of temperature. While the skin may be kept active and stimulated with salt water or Turkish baths and frictions with alcohol, bay rum, simple oils or fats, the best procedure when available embraces radiant heat (Bier’s or Dowsing’s apparatus) combined with general oily massage of the skin. I have applied the high-frequency currents over the spine, as well as to the areas affected, with much benefit in four cases. The remedy selected on a totality of symptoms is most important and often accomplished marked results. In this connection the reader is especially referred to Ruta, Rhodo., and Rhus tox., although Arsen., Bryonia, Cannab. ind., Graphites, Hydrocot., and Opium have all been used successfully.