(Morphea; Keloid of Addison)
Symptoms. – Though rare, this form is more common than the diffused type. often the lesions of this variety are unobserved because they attract so little attention, being without general or subjective sensations. They vary in shape, appearing in patches, bands or in variations or combinations of these; the patch-like outline being the more common. In size they range from a small pea to an area as large as the palm of the hand. They may be single or multiple. The color varies from a dull white like that of an old cicatrix to a yellowish-white which has been likened to seasoned ivory. The patch is often surrounded by a narrow pink, violet or lilac-colored border consisting of aggregations of minute blood vessels which have become dilated. Occasionally the dilated vessels run irregularly on to the body of the patch. Unilateral distribution is the rule. The patches may appear upon the trunk, especially on the breast, on the extremities, neck, head and face, particularly in the region supplied by the fifth nerve. The affected skin feels like parchment or firm leather, according to its thickness and may be level, depressed below or slightly raised above the surrounding surface. The diseased areas are dry, sometimes fissured, occasionally contracted into folds and usually smooth from the obliteration of the normal lines and the absence of hair. Variations in the features of typical morphea may be seen in almost any case. For example; odd forms may occur in the shape of straight or curved bands, streaks or ribbons; the center of a patch instead of being white may be a yellow, brown, green or black color; the vascular border may be absent; the main portion of the lesion may be hyperemic; the patches may develop quickly and rapid evolution cause such marked atrophy that the wasting of the part is noticeable; and even exostosis and ulceration have been noticed. Absence of sweat has been noted in the affected area without loss of sensibility or disturbance of sensation beyond moderate itching and burning.
The duration of morphea varies from one to ten years. It may disappear spontaneously leaving no trace but usually there remains marked scarring. It rarely involves a large extent of surface and seldom are the onset and course both active or both chronic. The general health is usually undisturbed, although when it runs a rapid course there may be associated with it several internal complications. Subjective sensations are slight or absent in the average case.
Etiology and Pathology – Little is known about the possible causes beyond the fact that the nerve supply of the affected area is abnormal as to result in defective innervation. The disease is more common in young adults and children and more frequent in women than in men. While the neurotic temperament and anxiety, worry and depressing surroundings seem to be predisposing factors, very definite exciting causes have been noted as changes in temperature, and local irritation from collars, garters, traumatisms and medicinal applications.
Pathologically, there is a cell exudation around the glands and vessels, narrowing the caliber of the latter and diminishing the current of blood. When the blood supply is entirely cut off, atrophy results. When the interference with circulation is incomplete, partial atrophy associated with increase of connective tissue results in a hypertrophic lesion. The capillary dilatation and colored border of the typical anemic spot is caused by an increase of blood pressure in the collateral capillaries at the periphery of the lesion.
Diagnosis. – Vitiligo is an atrophy of the pigment only without any change in the texture of the skin and present a dead white color rather than the yellowish or ivory white of morphea. Keloid and the hypertrophic band forms of circumscribed scleroderma may look alike but the former is denser, more vascular and elevated and usually shows claw-like prolongations extending into the sound skin. It is well to remember that cases of mixed scleroderma have been recorded and although few in number, they serve to show the pathological and clinical unity of the diffused and circumscribed forms. In two of my cases, the diffused type developed first and was followed in a few months by patches of morphea.
Prognosis and Treatment. – The former is guarded although some patches may disappear spontaneously. It is more common to see these lesions arrested than cured. The treatment is the same as for the diffuse form, that is by the means of an indicated remedy, tonics, massage and electricity. The X-rays and radium have been used but with only fair success. Electrolysis employed in the same manner as in the removal of superfluous hairs (recommended by Brocq), plus the use of a mercurial plaster has given good results.