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| | Title: | What is Scleroderma? | | Article: | Scleroderma means hard skin. Skin becomes thick and
hard because of excessive formation of collagen.
Collagen is a protein that normally forms connective
tissue. Hair and oily follicles undergo atrophy, which
means that they just disappear. Scleroderma becomes
either a progressive systemic sclerosis or localized
sclerosis (morphea).
Scleroderma is a chronic skin disease with board-like
hardening and immobility another feature is the
visceral involvement. That means that scleroderma
affects lungs, esophagus, kidneys and heart. The
picture is the same as with the skin – hardening and
decreased mobility, plus function disorder. Function
disorders happen because majority of organs consists
of numerous membranes, covered by the functional
cells. Because of thickening those membranes do not
work well. Oxygen does not go well into the blood in
lungs; kidneys do not filter liquid wastes well. Heart
and esophagus stiffen and so on.
Sometime Scleroderma goes with calcinosis, Raynaud's
phenomenon, and telangiectasis (CREST syndrome).
Calcinosis means calcium deposits in the skin (sort of
small bony stones). Raynaud's is the periodical white
and blue changes of fingers toes, ears, and nose
because of cut off supply of blood (usually due to
cold or strong emotions or other factors).
Telangiectasis is formation of small spider–like marks
on your skin. These marks are dilations of small
capillary blood vessels.
Acrosclerosis and sclerodactyly accompany scleroderma.
Sclerosis means hardening in Greek. Acrosclerosis is
hardening of finger and toe tips, stiffness of the
skin of the fingers, atrophy of the soft tissue of the
hands and feet, and osteoporosis of the finger
phalanges
Sclerodactily is hardening of fingers the way that a
hand looks like a claw. Basically, acrosclerosis is a
synonym of sclerodactily.
Localized scleroderma (morphea) looks like indurated,
slightly depressed plaque of thickened whitish or
yellowish-white skin surrounded by a pinkish or
purplish halo. These skin lesions may occur at any age
and usually resolve after a few years.
Linear scleroderma is a type of localized scleroderma
with band-like indurated skin. The skin has atrophy
and hyper- or hypopigmentation. Pigmentation means
staining of skin as for example in a tan or mole.
Hyperpigmentaion is a spot of skin that is darker then
the rest of the skin surface. Hypopigmentation is the
opposite –skin that is paler than surroundings. Both
may be disfiguring. Also the linear scleroderma may
extent into deeper tissues and cause joint
contractures, limiting the range of motion. Coup de
saber means the linear scleroderma of the forehead and
scalp.
Localized scleroderma can present as plaque,
generalized, bullous, linear or deep.
The causes of localized scleroderma are spontaneous
mostly. Sometime there is a preceding injury, BCG
vaccination or radiotherapy. Localized scleroderma is
three times more common in women than in men. The
disease often follows for many years. This is a benign
disease with slow progression. Half of the patients
may recover for a while. Sometimes localized
scleroderma comes with no other symptoms but itching
and cosmetic concerns.
Systemic scleroderma divides on limited cutaneous and
diffuse cutaneous.
The causes of systemic scleroderma are spontaneous
mostly. Sometime there is a preceding exposure to
environmental toxins or infections by bacteria named
Borrelia.
Limited cutaneous systemic scleroderma shows skin
sclerosis of face, hands, feet, and forearms, or
nothing at all. It also may show pulmonary
hypertension (increased pressure in lung blood
vessels) later during the diseases, trigeminal
neuralgia (pain in the face in area of trigeminal
nerve), calcinosis, and teleangiectasias. If you look
under microscope you may find dilated capillary loops
of nail folds. Anticentromere antibodies happen in
70-80% of cases.
During diffuse cutaneous systemic scleroderma you may
find truncal and acral (far point) skin changes,
Raynaud's phenomenon within one year of onset of skin
changes, friction rub of tendons.
Onsets of interstitial lung disease, kidney problems,
severe gastrointestinal disease, and heart involvement
are early and grave. In the lab diffuse cutaneous
systemic scleroderma shows anti-DNA topoisomerase I
(anti-Scl-70) antibodies, with absence of
anticentromere antibodies. Under microscopy nail-fold
capillary look dilated and destructed. From quarter to
half of the patients with the systemic scleroderma may
die during first five years because of kidney, lung
and heart problems. Fortunately it is not very common
disease affecting one person in 10 000.
Some drugs may cause the scleroderma-like skin
changes. These drugs include:
Cancer chemotherapeutics
Tryptofan
Analgesics
Neurological drugs
Appetite suppressants
Vitamin K
Penicillamine
Interferon,
Cocaine
Beta- blockers
Fosinopril
Morphine,
Ergot
Pentazocin
Ketobernidone | | Author: | Aleksandr Kavokin, MD, PhD | | System: | Skin | | Subject: | Scleroderma | | Abstract: | Scleroderma is a chronic skin disease with board-like
hardening and immobility another feature is the
visceral involvement. | | Website: | www.kavokin.com | | Time: | 18:29 | | Reference: | www.rdoctor.com | | Reference 2: | |
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©2006 Kavokin.com
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Articles by Aleksandr Kavokin, MD, PhD
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