Linear scleroderma is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound.
Abstract. The "neck sign" consists of ridging and tightening of the skin of the neck on extending the head. In this study it was found to be positive in over 90% of patients with scleroderma and negative in patients with primary Raynaud's disease and in control subjects. It is a useful diagnostic test for scleroderma.
Hard, thickening, or tight skin
Some people develop 1 or 2 patches of hard, thick skin. Others have widespread patches on their body. The hard, thick skin can feel anchored in place. If you have morphea (more-fee-uh), the most common type of scleroderma, the patches may not feel too hard.
Raynaud Phenomenon is the most common early symptom of systemic scleroderma. It is present at one time or another in about 90 percent of patients. It is most obvious in the fingers and toes but can also involve the ears, nose, and tip of the tongue.
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.
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.
Vasculitis: Vasculitis involves inflammation of blood vessels, leading to various symptoms depending on the affected organs. The diagnosis often requires imaging studies and biopsies, making it one of the more challenging autoimmune diseases to identify.
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.
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).
Clinically observable nail changes included longitudinal striations, thinning of the plate, absent lunula, "beaking," areas of hypopigmentation and hyperpigmentation, onychogryphosis, onychorrhexis, pterygium inver- sum unguis formation, and symmetrical onycholysis.
Gay Saget died from scleroderma, an autoimmune disease where excess collagen causes tissue to lose its elasticity, in 1994. She was 47.
Researchers suspect that exposure to some environmental factors, such as some chemicals, may trigger scleroderma. Immune system changes. Abnormal immune or inflammatory activity in your body triggers cell changes that cause the production of too much collagen.
Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose. After a thorough physical exam, your healthcare professional may suggest blood tests to check for elevated levels of certain antibodies made by the immune system.
Scleroderma patients frequently experience skin discoloration and/or hardening, skin tightening around the mouth, finger lesions, and in some cases digit amputation.
Actor Ashton Kutcher's rare autoimmune condition, vasculitis, is a disorder characterized by inflammation of the blood vessels that can be life-threatening.
Asherson's syndrome is an extremely rare autoimmune disorder characterized by the development, over a period of hours, days or weeks, of rapidly progressive blood clots affecting multiple organ systems of the body.
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.
The symptoms of scleroderma vary, depending on the type of scleroderma and which parts of the body are affected. Symptoms of localized scleroderma include: Reddish, oval patches on the trunk, legs, arms, or chest that may be itchy and/or tender.
Like many autoimmune diseases, systemic sclerosis, or scleroderma, can be a challenge to diagnose because its symptoms are varied, can range from mild to debilitating, and often resemble those of other conditions, such as systemic lupus erythematosus and rheumatoid arthritis.
This article aims to provide a comprehensive review of the more common cutaneous fibrosing disorders which mimic scleroderma. Specifically, the disorders of scleredema, scleromyxedema, eosinophilic fasciitis, pansclerotic morphea, nephrogenic systemic fibrosis and diabetic chieroarthropathy will be discussed.
REFLUX/HEARTBURN: eat small frequent meals to avoid overfilling your stomach; avoid eating within 2-3 hours of bedtime; avoid foods that may aggravate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli, raw ...
Thin lips are not only a common cosmetic concern in patients with scleroderma, a condition that involves tightening and hardening of the skin among other effects on internal organs, but also can impact a person's ability to chew, swallow, and sleep.