Which dermatologic findings are characteristic of scleroderma?

Updated: Nov 13, 2018
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

The skin of the hands may be edematous or swollen early in systemic sclerosis and the patient may initially report these changes as puffiness. This edematous stage precedes the indurated sclerotic stage. Slow progression of the sclerotic phase is associated with a better prognosis, whereas a rapid progression of cutaneous sclerosis indicates a worse prognosis and more extensive and severe visceral organ involvement with an increased risk of renal crisis or interstitial lung disease and higher mortality.

In the sclerotic phase, the skin appears tight and shiny (see image below), with a characteristic loss of hair, decreased sweating, and loss of the ability to make a skin fold. This process of skin thickening usually begins distally on the fingers (sclerodactyly; see image below) and progresses proximally in a continuous symmetrical fashion.

Tightening of the skin in the face, with a charact Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles.
Sclerodactyly with digital ulceration, loss of ski Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.

Other skin findings are as follows:

  • · Reduced oral aperture (microstomia) caused by perioral fibrosis (assessed by measurements of the incisor-to-incisor distance)

  • Skin pigmentary changes include a salt-and-pepper appearance, with areas of hyperpigmentation and hypopigmentation, or an overall appearance of darkened skin not related to sun exposure (see image below)

    Anterior chest demonstrating salt-and-pepper hypop Anterior chest demonstrating salt-and-pepper hypopigmentation and diffuse hyperpigmentation in a white woman.
  • Telangiectasias are dilated vessels located just beneath the dermis on any skin area, but they are most obvious in the face, hands, and anterior chest; occasionally, telangiectasias may occur in mucosal surfaces and cause either obvious or occult bleeding

  • Calcinosis may develop in the fingers and extremities (see image below), most commonly in the finger tips, the extensor surface of the forearms and in the prepatellar regions; however, any area of the body can be affected. Occasionally, large calcium deposits with the appearance of tumoral calcinosis may occur

    A radiograph of the distal digits demonstrating ca A radiograph of the distal digits demonstrating calcinosis and distal phalanx reabsorption (acral osteolysis).

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