...less medical jargon in a 'Quick Glance' format!
Linear Scleroderma, also known as bilateral scleroderma, is characterized by a highly visible band or bands of thick, tight skin on the arms, legs, chest, stomach, back, buttocks or face. It is often considered a pediatric disease as it usually manifests itself within the first 10 years of life.
When affecting a limb, this disease can cause arms and legs to develop at noticeably different rates. It is not uncommon for the disease to extend down to the bones and muscles underneath it, thus damaging their structure, limiting motion of affected joints, and interfering with normal growth.
When linear scleroderma occurs on the face, it is often referred to as en coup de saber, a French term for “a strike of the sword,” due to its appearance. Linear scleroderma may be disfiguring, but is not life threatening and rarely progresses to systemic scleroderma.
Symptoms:
In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone. Other signs and symptoms may include:
Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress
Stiffness or pain in your joints and curling of your fingers
Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in your intestine
Sores over joints, such as your elbows and knuckles
Puffy hands and feet, particularly in the morning
Causes:
There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A2 and TGF-β1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.
One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune reaction to what is perceived as "foreign" material.
A distinct form of scleroderma and systemic sclerosis may develop in patients with chronic renal failure. This entity, nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis, has been linked to the exposure to gadolinium-containing radiocontrast.
Treatment:
No treatment, medication or ointment has been proven by large-scale placebo-controlled studies to alter the course of this disease. A placebo-controlled study is still necessary to confirm the methotrexate/corticosteriod therapy; and, by any means, these are toxic therapies, particularly in children.
