Scleroderma
literally means "hard skin." Scleroderma is one of a group
of arthritic conditions called connective tissue
diseases.
The cause for scleroderma
is unknown. It's more common in women than in men
and more common in adults than in children.
Scleroderma can run in families, but in most cases
it occurs without any known family tendency for the
disease. It's not considered contagious or
cancerous.
Signs and 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 (Raynaud's phenomenon)
-
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 the intestine
-
Sores over joints,
such as your elbows and knuckles
-
Puffy hands and
feet, particularly in the morning
Causes
Scleroderma results from an overproduction and accumulation of collagen
in body tissues. Collagen is a fibrous type of protein found in
connective tissues, including your skin.
Although doctors aren't sure what prompts this abnormal collagen
production, the body's immune system appears to play a role. For unknown
reasons, the immune system turns against the body, producing
inflammation and the overproduction of collagen. In addition to its
effects on your skin, scleroderma affects tiny blood vessels and can
affect almost every organ.
Doctors classify scleroderma into different subsets:
Diffuse cutaneous systemic sclerosis
This
type affects the skin of your fingers, hands, arms, legs, face, neck and
trunk. Internal organs such as your lungs, heart, gastrointestinal tract
— including your esophagus — and kidneys may be affected. It can hinder
the functions of your digestive system, create respiratory problems and
cause kidney failure. Untreated, systemic scleroderma may be fatal
within several years of onset.
Limited cutaneous systemic sclerosis
This
type involves the skin of your fingers, lower arms and legs, face and
neck. A variation is called CREST syndrome. CREST stands for these
conditions:
-
Calcinosis
— calcification in the skin
-
Raynaud's
phenomenon
-
Esophageal
dysfunction — such as reflux or difficulty in swallowing
-
Sclerodactyly
— hardening of the skin of the fingers or toes
-
Telangiectasia
— dilatation of tiny blood vessels, particularly of the skin
Localized scleroderma
This
type affects mainly your skin and deep tissues below your skin. It
includes the following subclassifications:
-
Morphea.
In this form, oval-shaped thick patches appear on your skin — white
in the middle, with a purple border. These patches are most likely
to occur on your torso, but they can also appear on your arms, legs
or forehead.
-
Linear
scleroderma.
This form results in bands or streaks of hardened skin on one or
both of your arms or legs, or on your forehead.
Overlap syndrome
This
is diffuse or limited systemic sclerosis with features of one or more of
the other connective tissue diseases. Mixed connective tissue disease (MCTD)
is an overlap syndrome with features of scleroderma, systemic lupus erythematosus, polymyositis and rheumatoid arthritis, and with the
presence of an antibody produced by your immune system that is directed
against cellular proteins in your body.
Undifferentiated connective tissue disease
This
has features of systemic sclerosis, but there are no definite clinical
or laboratory findings to make a definite diagnosis.
Sine scleroderma
Some
doctors may describe one variation of scleroderma as sine scleroderma,
which can be similar to either limited or diffuse scleroderma, the
difference being that this form doesn't affect your skin. Sine is
the Latin word for "without."
Scleroderma -
systemic sclerosis
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