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February is Raynaud's Awareness Month: Raynaud's is a condition in which the blond flow to the fingers, toes and sometimes ears and nose is interrupted. An 'attack' can be brought on by exposure to cold of any kind {eg, weather or objects) or a slight change in temperature. Anxiety and smoking can make the condition worse.

There are varying degrees of the disease. In some cases it is hereditary and women are nine times more likely to be affected than men.

People who work with vibratory tools can sometimes develop a condition call vibration white finger, which is the industrial form of Raynaud's.

Sometime during the late 1850's a young French girl of 27 years noticed that the fingers of both hands had suddenly become pale and cold. Over the next few weeks the tips of some of her fingers shrivelled up and turned black, causing her great pain and discomfort. The doctor who attended her was an equally young man called Maurice Raynaud. He was perplexed, for he was faced with a patient who, had spontaneously developed gangrene of her fingers with no apparent cause. She seemed healthy, there was a good pulse in her wrists indicating a good blood supply to her hands, yet her fingers looked starved of blood and the tips of her fingers were gangrenous. His medical teaching told him that gangrene was due to either blockage of a main artery In a limb, diabetes, typhoid, or poisoning by a fungus called "ergot", which grows on mouldy rye. Clearly his patient did not tit Into any of these categories, but she did have a curious history of her fingers turning odd colours whenever she was cold or under stress. He decided that the colour changes must have been due to some sort of intermittent blockage of the blood supply and that the spontaneous gangrene of her fingertips was in some way connected.

Over the next few years Raynaud saw similar patients and discussed the problem with colleagues who had also come across the condition. Before long he had collected the case histories of some 23 patients whose fingers, toes, or sometimes face and ears changed colour in the cold. Some of the patients had also developed areas of gangrene on the affected parts. He was, however, still unsure as to the cause of the problem.

At around the same time another French doctor called Claude Bernard discovered that arteries, those vessels which carry bright red fresh blood all around the body, are supplied by nerves which make them constrict, that is, when the nerves were activated the arteries would decrease In calibre and carry less blood. The nerves in question were called the sympathetic nerves. Here at last was Raynaud's answer I The sympathetic nerves must be overactive, he thought, making the arteries so narrow that not enough blood can get through to the extremities to keep them alive. When the condition is severe enough gangrene develops, but In its milder form the affected areas intermittently turn white or blue and finally red when the blood eventually returns. In essence the fingers and anywhere else affected were being strangled or asphyxiated due to lack of blood! He published his thoughts in the form of a thesis In 1862 called "Del'asphyxie locale et de la gangrene symetrique des extremites" translated as "On Local Asphyxia and Symmetrical Gangrene of the Extremities", inevitably, the condition came to be known as Raynaud's Disease. {Now called Raynaud's phenomenon or simply Raynaud's).

During n Raynaud's attack, the affected areas become white and dead-looking in appearance; they then turn blue and finally red and start to burn. This is occasionally accompanied by numbness, considerable pain or a tingling sensation. Prognosis can be difficult to determine and other important factors to take into consideration are the use of certain drugs such as betablockers (which can cause cold, white hands) and the patients occupation. Much confusion reigns as to the correct terminology and more recently the word 'disease' has been dropped, the condition simply being called Raynaud's or Raynaud's phenomenon,

Raynaud's can be subdivided Into primary or secondary. Anyone of any age can develop Primary Raynaud's which occurs spontaneously without any underlying condition being present. It can be hereditary In which case It is usually fairly mild. Woman are affected nine times more than men. Secondary Raynaud's is less common and is associated with underlying diseases such as scleroderma (systemic sclerosis), systemic lupus, erythematosus, Sjogren's syndrome and rheumatoid arthritis. Secondary Raynaud's is more serious than Primary Raynaud's, so early and accurate diagnosis is essential.

There are two tests which are critical at an early stage in defining whether Raynaud's is primary or secondary. One is a blood test to look for antinuclear antibodies. If the test is abnormal then the patient should be referred to a specialist centre. The other test would be to look at the capillaries at the base of the nail with an opthtalmoscopy to look for abnormal patterns.

Although Primary Raynaud's is common, only a small proportion of people with Raynaud's develop scleroderma.

The word scleroderma comes from two Greek words sclero: meaning hard and derma: meaning skin. Scleroderma is a disease of the immune system, the blood vessels and connective tissue. In this condition the skin, usually on the hands and feet becomes stiff, tight and shiny. This is because of swelling and then thickening of the connective tissue which becomes fibrotic or scarred. Internal organs can also be affected in a similar way.

There are different types of the condition; diffuse cutaneous systemic sclerosis (dcSSc), limited cutaneous systemic sclerosis (IcSSc) and localised scleroderma (of which there are two types: morphea and linear.

Diffuse Cutaneous Systemic Sclerosis (dcSSc)

Shortly after the onset of Raynaud's the patient presents with skin changes (puffy or hidebound), has truncal skin involvement and 'creaking' tendons at Joints, e.g. wrists and elbows. Early lung disease, kidney, gastrointestinal and heart Involvement becomes evident in some patients. There are changes in the nailfold capillary and certain antibodies may be present in the blood. A specific marker of scleroderma, usually the diffuse form is 5c 1-70 which is present In 30% of patients.

Anyone with diffuse SSc needs to be checked frequently {at least every 6-12 months] in the first 5 years.

Limited Cutaneous Systemic Sclerosis (IcSSc)

Patients with limited SSc will have had Raynaud's for years, occasionally decades and there will be skin involvement to the hands, face, feet and forearms. These patients used to be classified as having CREST. In the early 1990's several physicians noted a group of features often seen when they examined patients with sceroderma (systemic sclerosis). They coined the term CREST syndrome.

This is now known as limited cutaneous systemic sclerosis. The word CREST stands for 5 symptoms which patients may experience:-

C--Calcinosis which is an accumulation of calcium below the outer layer of the skin.

R--Raynaud's phenomenon--a condition which the blood supply to the extremities, usually the fingers and toes, is temporarily interrupted.

E--Esophageal {gullet) involvement, causing difficulty in swallowing or indigestion.

s--Sclerodactyly is when the skin of the digits becomes thin, shiny, and leathery looking. Fingers and toes may become flexed and stiff,

T--Telangiectasia Is the appearance of small blood vessels near the surface of the skin. These can be seen on the fingers, palms, lips, face, tongue and chest wall. Patients with limited disease need checking every 6-12 months, depending on the stability of the disease.

Other problems

Scleroderma patients may have problems with dryness of the mouth and eyes due to a decrease in secretion from the salivary and tear glands. They may experience difficulty in swallowing, bloating or abdominal pain, tiredness, lack of energy, general weakness, weight loss, aching muscles, Joints and bones. Involvement of the lungs, heart and kidneys may also occur.

Localised Scleroderma

There are two types of localised scleroderma. Patients may be subject to either or a mixture of both.

Morphea

Morphea affects the skin, beginning with an inflammatory tage, followed by the development of one or many slowly enlarging patches or plaques. These plaques are usually oval in shape but vary in size and colour and may enlarge or shrink, sometimes disappearing spontaneously.

Linear Scleroderma

Linear scleroderma usually develops in childhood and can affect the growth of a limb. It Is a form of localised scleroderma, which starts as a band-like thickening of skin, usually limited to one area, such as an arm, leg or Forehead (en coup de sabre). Unlike morphea, linear scleroderma tends to involve layers of tissue below the skin. It can sometimes affect the muscles and bones and finally the mobility of the underlying joints.

Symptoms of Scleroderma

Symptoms differ in severity depending on the type of scleroderma. Raynaud's is usually the first symptom, which may be followed by difficulty swallowing. If the lungs are involved the patient may feel breathless at times.

There's no cure for Raynaud's or scleroderma, but prescribed medication, exercise, stress control, keeping warm and giving up smoking can all help to manage them. It is thought that avoiding standing still for long periods in cold or draughty areas and keeping active to maintain body warmth, wearing tights or "long johns" under trousers can all help. Shoes should be water resistant and have thick soles and should not be too light as this causes restriction to circulation.limited cutaneous systemic sclerosis. The word CREST stands for 5 symptoms which Patients may experience:

The Institute thanks the Raynaud's and Scleroderma fat supplying this information. The Raynaud's & Scleroderma Association offers support and advice to patients and their families and assists in the welfare of the chronically sick and those with disabilities resulting from the conditions. It alms to raise awareness and to raise funds for welfare projects and for research into the conditions. Call 0300 917 2494 for a free information pack or go to www.raynauds.org.uk
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Title Annotation:Awareness News; Raynaud's disease; Maurice Raynaud
Publication:Podiatry Review
Geographic Code:4EUUK
Date:Jan 1, 2013
Words:1587
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