The causes are obscure. The disease is rare, and is encountered oftener in women than in men, and occurs usually in early adult or middle life. Sudden changes of temperature, exposure to wet or cold, and violent impressions on the nervous system have been cited as causes. The anatomy of the disease has been studied by various observers, but with different results, in the majority of cases slight structural changes only having been found. Both the true skin and the subcutaneous connective tissue are the seat of the process, showing a marked increase of the connective tissue, with thickening and condensation of the fibres. The disease may be viewed as a tropho-neurosis. The diagnosis, as a rule, presents no difficulty. From morphoea, to which it is closely allied, it may be distinguished by its tendency to involve large areas, occupying sometimes the greater portion or the whole of the integument, whereas morphoea usually appears in smaller lesions. Scleroderma manifests itself diffusely and without lines of demarcation; morphoea is circumscribed, and in its early stage is surrounded by a pinkish border. Scleroderma is always characterized by stiffness or hardness, whereas morphoea is usually soft or firm. In scleroderma the skin is merely rigid or hard in the beginning, whereas in morphoea there is hyperæmia and only slight induration.
Concerning the treatment of this disease there is but little to be said. Constitutional remedies, such as arsenic, quinine, and cod-liver oil, together with the employment of stimulating oily or fatty applications, frictions, and electricity are indicated, though it is difficult to state their intrinsic value. The course and termination of the disease varies. In some cases spontaneous involution sets in sooner or later, while in other instances the process continues to progress, and lasts throughout life.
Morphoea.
Morphoea, formerly known as keloid of Addison, is characterized by one or more rounded, ovalish or elongate, coin-sized patches, which, as a rule, are circumscribed and clearly defined. At first they are hyperæmic and pinkish, becoming as the process advances pale yellowish or whitish, with a faint pinkish or lilac border made up of very minute injected capillaries. The patch may be slightly elevated or puffed in the beginning, but later is on a level with the surrounding skin, or even somewhat depressed. When typically developed it is either soft or firm to the touch, or, more rarely, leathery or brawny. The surface is usually smooth, and may be shining and have an atrophic appearance. Not infrequently it resembles in color and in look a piece of cut bacon or ivory laid in the skin. Around the patch there is usually, in addition to the hyperæmic border, more or less diffuse, mottled yellowish or brownish pigmentation. The disease exhibits no disposition to symmetry, but not infrequently it manifests itself over nerve-tracts. The regions commonly invaded are the face, neck, chest, mammæ, back, abdomen, arms, and thighs. The lesions pursue a variable though usually chronic course, lasting, as a rule, years. There is always a marked tendency to varied atrophic changes, which in most cases appear early, the skin becoming thin, shrivelled, or parchment-like, later being bound down to the tissues beneath, forming cicatriform, keloidal lesions, which may cause contraction and deformity, with, in some cases, wasting and general atrophy, more particularly of the extremities.
In addition to the usual characteristic circumscribed patches described, there may exist distinctly atrophic lesions consisting of small pit-like depressions resembling scars; also, reddish or bluish, tortuous, short or long, large and minute, dilated, superficial cutaneous blood-vessels and telangiectases, together with smooth, glazed, whitish, slightly-depressed spots or grooved streaks—true maculæ et striæ atrophicæ. Accompanying these various lesions there is usually considerable diffuse or patchy yellowish or brownish pigmentation. The process in some cases is simple as regards the lesions, but not infrequently it is complex, being characterized, as indicated, by a variety of lesions in different stages of evolution. The course is chronic, extending in the majority of cases over years. The disease in some cases eventually tends to spontaneous recovery; and this is all the more remarkable considering that atrophy has existed. The disease is met with more frequently in females than in males. Impaired nerve-power is without doubt the important factor in its production. Concerning the relation of morphoea to scleroderma, it may be said that these affections are closely allied, and that they may occur together. The pathological anatomy of the characteristic patches varies with the stage of the disease. In the early stages there is shrinkage or atrophy of the papillary layer, with condensation of the connective tissue of the corium. Crocker further noted marked cell-infiltration around the sebaceous glands, hair-follicles, and vessels, and in the later stages the transformation of these cells into fibrillar tissue, its contraction, and the consequent obliteration of blood-vessels, with atrophy of the sebaceous and sweat glands.
Morphoea is to be distinguished from scleroderma, from vitiligo, and from the anæsthetic patches of leprosy. In appearance morphoea and leprosy possess features in common, and it is probable that they are both due to the same cause—namely, perverted innervation. As a rule, no difficulty will arise in the diagnosis, for the reason that in leprosy other symptoms of that disease will almost invariably be present.
To be viewed as a variety or form of morphoea, we may mention hemi-atrophia facialis, or unilateral atrophy of the face, which affection consists of a variable degree of atrophy of the skin and deeper structures, the cutaneous lesions being the same as those in morphoea. The neurotic origin of the disease in this case is plain.
A general tonic treatment, with the long-continued use of such remedies as arsenic, quinine, cod-liver oil, iodide of potassium, and electricity, is called for, most reliance being placed upon arsenic. Good results sometimes follow its administration. The prognosis should always be guarded.
Elephantiasis.
Elephantiasis, or elephantiasis arabum (also called pachydermia, Barbadoes leg, elephant leg), is a chronic hypertrophic disease of the skin and subcutaneous tissue, characterized by enlargement and deformity of the part affected, accompanied by lymphangitis, swelling, oedema, thickening, induration, pigmentation, and more or less papillary growth. The legs and genitalia, especially the former, are favorite localities for its development; about the latter, the penis, scrotum, and clitoris are most frequently involved. It begins with an inflammation of the parts, erysipelatous in character, attended with febrile disturbance, swelling, pain, heat, redness, and lymphangitis. The inflammation may have its starting-point in a local lesion, as a wound or scar, or, as is usually the case, manifests itself without any apparent cause. Similar attacks occur more or less frequently, after each of which the part remains increased in size. After a year or longer, during which time repeated attacks may have taken place, considerable increase in size is noted: the part is swollen, oedematous, and hard, and the skin hypertrophied, fissured, pigmented, and the papillæ enlarged and prominent. Later, the hypertrophy becomes still more marked; the part is often enormously enlarged and swollen, the skin rough, fissured, and warty. In Eastern countries the disease assumes huge proportions. Eczematous inflammation may coexist and complicate the appearance. The fissures may be slight or large and deep, the normal lines and folds of the surface exaggerated, with more or less maceration of the epidermis taking place, especially about the folds. Ulcers sooner or later tend to form, developing usually from varicose veins, while scales and crusts may also be present. Pain varies, being usually marked during the inflammatory attacks.