(Synonyms: Sclerema; Scleriasis; Dermatosclerosis; Morphoea; Keloid of Addison.)

What is scleroderma?

Scleroderma is an acute or chronic disease of the skin characterized by a localized or general, more or less diffuse, usually pigmented, rigid, stiffened, indurated or hide-bound condition.

Morphoea, by some formerly thought to be a distinct affection, is now believed to be a form of scleroderma; as typically met with it is characterized by one or more rounded, oval, or elongate, coin- to palm-sized, pinkish, or whitish ivory-looking patches. In some instances such patches are seen in association with the more classic type of scleroderma just defined.

Describe the symptoms of ordinary scleroderma.

The disease may be acute or chronic, usually the latter. A portion or almost the entire surface may be involved, or it may occupy variously sized and shaped areas. The integument becomes more or less rigid and indurated, hard to the touch, hide-bound, and in marked cases immobile. Œdema may, especially in the more acute cases, precede the induration. Pigmentation, of a yellowish or brownish color, is often a precursory and accompanying symptom. The skin feels tight and contracted, and in some instances numbness and cramp-like pains are complained of.

Describe the variety known as morphoea.

The patches (one, several, or more), occurring most frequently about the trunk, are in the beginning usually slightly hyperÆmic, later becoming pale-yellowish or white, and having a pinkish or lilac border made up of minute capillaries. They are, as a rule, sharply defined, with a smooth, often shining and atrophic-looking surface; are soft, fine or leathery to the touch, on a level or somewhat depressed, and appearing not unlike a piece of bacon or ivory laid in the skin. Occasionally the patches are noted to occur over nerve-tracts. The adjacent skin may be normal or there may be more or less yellowish or brownish mottling. The subjective symptoms of tingling, itching, numbness, and even pain, may or may not be present.

What is the course of the disease?

Sooner or later, usually after months or years, the disease ends in resolution and recovery, or in marked atrophic changes, causing contraction and deformity. As a rule, the general health remains good.

State the causes of scleroderma.

The condition is to be considered as probably of neurotic origin. Exposure and shock to the nervous system are to be looked upon as influential. It is a rare disease, observed usually in early adult or middle life, and is more frequent in women than in men.

What is the pathology?

In typical and advanced cases both the true skin and the subcutaneous connective tissue show a marked increase of connective tissue-element, with thickening and condensation of the fibers.

Is there any difficulty in reaching a diagnosis in scleroderma?

As a rule, no. The characters—rigidity, stiffness, hardness, and hide-bound condition of the skin—are always distinctive.

The peculiar appearance, the course and character of the patches, of morphoea are quite distinctive.

Give the prognosis of scleroderma.

It should always be guarded. In many instances recovery takes place, whilst in others the disease is rebellious, lasting indefinitely. The prognosis of the variety known as morphoea is less unfavorable than general scleroderma, and recovery more frequent.

What is the treatment of scleroderma?

Tonics, such as arsenic, quinia, nux vomica, and cod-liver oil; conjointly with the local employment of stimulating, oily or fatty applications, friction, and electricity. RÖntgen-ray treatment is often of value, more especially in the morphoea type.