Signs and Symptoms
The manifestations of scleroderma are variable and dependent upon the extent of systemic involvement. For example, localized scleroderma may affect only the fingers and cause moderate hardening of the skin, whereas severe systemic sclerosis can compromise internal organs such as the lungs and kidneys and be life threatening. Some of the more prominent features of scleroderma include:
Skin manifestations of scleroderma include puffy and swollen fingers, hands, and toes, along with thickening and hardening of the skin, which can also affect the face and trunk. The areas of the skin affected often appear shiny and hair loss occurs in affected regions as well; skin may also appear abnormally light or dark. Tightening of the skin on the face can give a mask-like appearance. Sometimes, patients develop ulcers on their fingertips or toes, which can lead to scarring. In addition, small blood vessels just beneath the top layer of the skin may become dilated and more visually pronounced, called telangiectasias (Gaby 2006; Chatterjee 2010; A.D.A.M. 2013).
Raynaud's phenomenon is present in up to 95% of patients with scleroderma (Hinchcliff 2008; Simonini 2000; NIAMS 2010). Raynaud’s phenomenon is a complex vascular disorder in which blood vessels, particularly those in the fingers and toes, or rarely the tongue, nose, ears, lips, or nipples, overreact to cold temperature or emotional stress (Mayo Clinic 2011; Herrick 2012; Martínez 2011). The blood vessels constrict (ie, vasoconstriction), reducing blood flow to the affected extremity. This results in numbness while blood flow is reduced and tingling and/or pain as blood flow returns to the affected area (Herrick 2012; Martínez 2011). These attacks may last from minutes to hours, and the intensity of discomfort may vary from mild to severe (Malenfant 2011; NCBI 2011; Martínez 2011). In severe cases, prolonged oxygen deprivation can lead to tissue death in the affected extremities (Herrick 2012). More information is available in the Raynaud’s Phenomenon protocol.
Internal Organ Involvement
Systemic sclerosis can affect multiple internal organs. However, the disease does not progress in exactly the same way in all patients. Some significant manifestations include (Balbir-Gurman 2012):
- Symptoms due to lung involvement include dry cough and shortness of breath (A.D.A.M. 2013). Cough is usually correlated with the degree of fibrosis in the lungs (Theodore 2012). Lung function tests are abnormal in as many as 80% of scleroderma patients (Jimenez 2012).
- Heart and cardiovascular complications are caused by scarring and weakening of the heart muscle, and include myocarditis and arrhythmia (NIAMS 2012). In addition, patients with systemic sclerosis have significantly increased risk of atherosclerosis, heart attack, stroke, and peripheral vascular disease compared to healthy individuals (Man 2013; Au 2011). Thus, individuals with systemic sclerosis are also encouraged to review Life Extension’s Atherosclerosis and Cardiovascular Disease protocol.
- A serious complication, when the kidneys are affected, is scleroderma renal crisis. This may lead to abrupt onset high blood pressure and progressive kidney failure (NIAMS 2012).
- Digestive problems include difficulty swallowing, esophageal reflux, bloating, diarrhea, constipation, and fecal incontinence (A.D.A.M. 2013).