What to expect: Your doctor will closely examine several areas of your skin where you may have thickening. Usually, they will lightly pinch your skin between two fingers or a finger and a thumb to gauge its thickness on a score of zero (soft, pliable skin with no thickening) to three. This pinch will not be painful.
You can quickly check for dehydration at home. Pinch the skin over the back of the hand, on the abdomen, or over the front of the chest under the collarbone. This will show skin turgor. Mild dehydration will cause the skin to be slightly slow in its return to normal.
Approximately 95% of patients with SSc have antinuclear antibodies (ANAs), 3 so testing for ANAs is an initial step in disease assessment. Subsequent testing for SSc is determined by the ANA patterns observed (eg, homogeneous, centromere, nucleolar, and speckled nuclear patterns or reticular/AMA cytoplasmic pattern).
Thickening and swelling of the fingers. Pale fingers that may become numb and tingle when exposed to cold or stress, known as Raynaud's phenomenon. Joint pain. Taut, shiny, darker skin on large areas, which can cause problems with movement.
The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.
All patients with scleroderma have certain shared features of the illness which include: Progressive scarring of blood vessels beginning in the fingers but with risk of extending to internal organs. Scarring of tissues outside the blood vessels including skin thickening and lung involvement.
This form of scleroderma can occur at any age but is most common in women of childbearing age. It occurs in around 30 persons per million population per year and there are an estimated 125,000 active cases in the United States and perhaps 2.5 million worldwide. It is the most fatal of all the rheumatologic diseases.
Skin turgor is generally accepted as part of the clinical assessment of hydration status in children being a quick, non‐invasive test that can be performed at the bedside.
Skin manifestations
Scleroderma: Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Scleroderma: Anterior chest demonstrating salt-and-pepper hypopigmentation and diffuse hyperpigmentation in a white woman.
Tocilizumab. Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. It's an IL-6 inhibitor, meaning it targets and blocks an inflammatory protein or “cytokine” called interleukin-6 (IL-6).
In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud's and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.
Survival rates for lupus are 85–90% during the first 10 years. For systemic scleroderma, the 5-year survival rates are up to 80% . However, if pulmonary arterial hypertension is present, the 2-year survival rate is less than 50%.
Since vitamin D has immunomodulatory, cardioprotective, and antifibrotic properties, it could potentially interfere with SSc pathogenesis. Suboptimal vitamin D levels are classically recognized in scleroderma, irrespective of clinical and serological phenotype.
Gay Saget died from scleroderma, an autoimmune disease where excess collagen causes tissue to lose its elasticity, in 1994. She was 47.
Use healthy oils, such as olive oil or vegetable oil. Reduce or eliminate trans-fatty acids, found in commercially-baked goods such as cookies, crackers, cakes, French fries, onion rings, donuts, and processed foods. Avoid caffeine, alcohol, and tobacco. Drink 6 to 8 glasses of filtered water daily.
The scleroderma neck sign, as described by Barnett, is a visible and palpable tight band over platysma in the hyperextended neck. A recent survey of 76 patients with scleroderma revealed that more than 90% had the scleroderma neck sign.
Morphea, also called localized scleroderma, is a rare inflammatory skin condition that can also affect the subcutaneous tissues. Morphea encompasses multiple variants with different outcomes ranging for small isolated benign skin lesions to aggressive lesions, which can cause significant deformities.
People with scleroderma are much more likely to have two other conditions: Raynaud's syndrome and Sjögren's syndrome. Reynaud's syndrome affects small blood vessels in your fingers and toes (your digits).