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Do you suffer Scleroderma ?

Posted Sep 11 2009 4:59pm

Title: Do you suffer Scleroderma? (Available from )
Author: Aleksandr Kavokin, MD, PhD,
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, linearor 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 ofthe 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
Scleroderma Foundation
MedlinePlus: Scleroderma
Check scleroderma symptoms
Scleroderma Research Foundation

Keywords: scleroderma, skin, Skin hardening , rash, butterflyrash, kidney, pain, autoimmune

About the author:Aleksandr Kavokin, MD, PhD, http://www.kavokin.com
Aleksandr received his MD from RussianState Medical University. Later he did his PhD in Immunology andAllergy. He did research in Cancer Biology at Medical Universityof South Carolina, 5 years of research in Cardiology at YaleUniversity School of Medicine, had internship in General Surgeryat Graduate Hospital in Philadelphia

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