Detailed Symptoms of Behcets Disease.

 

The symptoms of Behcet's were documented way back in 500 BC, by the well known physician of ancient Greece, Hippocrates, and Behcet's was known under various names in ancient China and in the late 19th and early 20th century in Europe.

 

It wasn't until 1937 however that the symptoms were given a name. As is the case of most discoveries, the symptoms and disease were named after the defining doctor or physician. In this case it was Hulusi Behcet, (1889-1948), a professor of Dermatology in Istanbul.

 

So it could have been called Hippocrates disease, now there's a thought.

 

Behcet's is a rare, chronic, lifelong disorder that involves inflammation of the blood vessels throughout the body.

 

Behcet's disease generally begins when patients are in their 20's or 30's, although all age groups have been documented with the disease. Behcet's disease is also known as the silk route disease, as the frequency of Behcet's cases are more prominent along this route. The silk route takes you from central Europe to China. However because of migration the Behcet's can be found in populations worldwide. Behcets Organisation Worldwide (www.behcets.org) currently helps people in over 30 countries!

 

As mentioned above, Behcet's could well have been called Hippocrates disease, and that may be more apt, because the disease can have periods of being dormant, called remission, and periods of activity called 'flares' and that the symptoms can appear individually, making it seem like you are a hypochondriac.

 

As mentioned, these periods can be called flares and remissions. Flares being when the disease is active, remission when the disease is inactive (dormant).

 

This can be terribly frustrating, as each patient may have the symptoms constantly, periodically and the symptoms can also be mild or severe.

 

Depending on which applies to you will depend on how much your quality of life is affected. (see 'How Behcet's may affect you' ).

 

The symptoms of Behcet's Syndrome (also called Behcet's disease), depend on the area of the body affected. Behcet's syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the bodies tissues. Behcet's Disease can also affect the veins that take the blood back to the lungs to replenish the oxygen content. Other areas of the body that can be affected by the inflammation of Behcet's Disease include the back of the eyes (retina), brain, joints, skin, and bowels.

 

The mouth and genital ulcers are generally painful and recur in crops. They can range in size from a few millimetres to 20 millimetres in diameter.

 

Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. It is very important for patients to have this sensitive area monitored by an eye specialist (ophthalmologist).

 

If the arteries become inflamed (arteritis) in patients with Behcet's Disease, it can lead to death of the tissues whose oxygen supply depends on these vessels. This could cause a stroke if affecting the brain, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis), the inflammation can involve large veins that develop blood clots, which can loosen to cause pulmonary embolism.

 

Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever. This can cause damage to nervous tissue and lead to weakness of body areas.

 

Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints in patients with Behcet's Disease.

 

The skin of patients with Behcet's Disease can develop areas of inflammation which spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behcet's Disease develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles. Genital ulcerations can occur on the penis, scrotum, labia, vagina and cervix.

 

Ulcerations can occur at any location in the stomach, or large or small bowel in patients with Behcet's.

 

Now for the MEDICAL TERMINOLOGY of many symptoms commonly found in Behcets Disease:

 

(A)  Dermatological Symptoms --- As the term implies, this has to do with the skin.   Frequently found evidences of this are: 

 

      (1)  Erythema Nodosum --- An eruption of red-purple swellings especially on the legs.  It is most commonly found between the ages of 20 years and 50 years and affects women more often than men. 

 

      (2)  Papulopustules --- Small, solid, slightly raised areas of the skin and are usually less than half an inch (1 cm) in diameter and may be round or flat, with a smooth or warty texture.  They may be the color of surrounding skin or pigmented and usually contain pus. Pseudofolliculitis Acne that may or may not be indicative of actual infection within the body but most commonly the face or around hair follicles. 

 

      (3)  Purpura --- Any of a group of disorders characterized by purplish or reddish brown area or spots of discoloration of the skin, ranging in size from pin-head to an inch or so in diameter, visible through the skin and caused by bleeding within underlying tissues with smaller bleeding points being sometimes called "petechiae" and larger darker ones being called bruises of "ecchymoses".

 

     (4)  Spider Nevus or Nevi, Splinter hemorrhages, Spider Veins, Telangiectasia --- A discolored patch of skin that takes the form or a red, raised dot the size of a pin-head with small blood vessels radiating from this dot and represent the outward appearance of a dilated arteriole (small artery) and its connecting capillaries.  They may be singular or multiple and adjoining. 

 

     (5)  Pyoderma Gangrenosum --- Characterized by ulcers, usually on the legs, that turn into hard, painful areas surrounded by discolored skin. 

 

     (6)  Pathergy --- Trauma to the skin which results in over-reaction of the skin and appearing as severe bruising being the result of an injury. 

 

(B)  Joints and Cartilage --- Swelling and inflammation of the cartilage or connective tissues surrounding the bones and joints with knees a particular primary target, although this may occur in any joint of the body. 

 

(C)  Central Nervous System --- often referred to as "CNS" --- Involvement often seen or identified as: 

 

      (1)  Bacterial or Aseptic Meningitis --- Inflammation of the meninges or membranes covering the brain and spinal cord and usually results from

 

bacterial infection or inflammation by a variety of micro-organisms not of

 

a viral nature and is life-threatening and must have prompt and proper treatment. 

 

     (2)  Migraine Headaches --- Severe headaches lasting from 2 hours to days of no specific number and is usually accompanied by vision disturbance and/or nausea with and without vomiting.   Factors for these headaches may be stress related (anxiety, anger, worry, fear, excitement, depression, shock, over-exertion, routine changes, or climate changes), or food related (particularly dairy and cheese products, eggs, red wine, fried foods, citrus foods, or chocolate), or sensory related (bright light or glare, loud noises, and intense or penetrating smells).   These headaches are generally of two types:

 

           (a)   Common Migraine --- Pain develops slowly and sometimes mounting to a throbbing pain and is often, but not always, on one side of the head only and usually occurs with nausea and sometimes vomiting. 

 

            (b)  Classical Migraine --- Pain is preceded by a slowly expanding area of blindness surrounded by a sparkling edge that increases to involve up to 1/2 of the field of vision of each eye and lasts for about 20 minutes followed by a severe one-sided headache with nausea, vomiting, sensitivity of light, and other temporary neurological symptoms such as weakness in one half the body.   

 

     (3)  Papilledema --- Optic Disk edema (swelling of the head of the optic nerve) caused by a rise in pressure within the brain. 

 

     (4)  Paresthesia --- Altered sensations in the skin that causes numbness and tingling known as a "pins and needles sensation". 

 

     (5)  Motor Neuron Dusease or sometimes more commonly called ALS or Amyotrophic Lateral Sclerosis --- It is first noted as weakness in the hands and arms and may be accompanied by wasting of the muscles.

 

           Fasciculation, an involuntary quivering of small area of the muscle may also occur. Sufferers also report cramping and stiffness. Sometimes the weakness starts in the legs but in almost every case, all four extremities are seen involved. 

 

     (6)  Stroke --- Damage to part of the brain caused by interruption to its blood supply by blockage of a vessel containing thrombus (blood-clot) or leakage of blood outside of vessel walls as that of a perforation of the blood vessel as is common of an aneurism or ulceration of the blood vessel. 

 

     (7)  Memory Loss or Impairment --- The loss of the ability to memorize and/or recall information stored in memory and which may be short-term duration of seconds or minutes only or long-term and lasting for days to permanent memory failure. 

 

     (8)  Hypertension, known as Benign Intracranial --- Occurs when blood flow to and from the brain within the head is restricted causing too much pressure on the brain. 

 

     (9)  Depression --- Feelings of sadness, hopelessness, pessimism, and a general loss of interest in life combined with a sense of reduced emotional well-being.   Symptoms are usually shown in variable moods, loss of appetite, sleeping difficulty, loss of interest and pleasure in social activities, tiredness or fatigue, loss of concentration, movement either slowed or hyper as in anxious, agitated, or fidgety, suicidal thoughts, feelings of guilt or worthlessness, hallucinations or delusions (believing or envisioning something that is not real). 

 

   (10)  Psychosis --- A mental disorder in which the individual loses contact with reality which disturbs the ability to think, perceive, and judge clearly such that sufferers often do not realize they are sick and may be identified as schizophrenia, manic-depressive, organic brain syndrome or paranoia. 

 

  (D)   Gastro-Intestinal and Digestive Tract --- Frequently presented with ulcerations or lesions beginning in the oral cavity (mouth, tongue, throat, esophagus) and extending through every internal organ connected to and with the digestive process of food and beverages to the expulsion of those food remains called feces thru the colon and anus. 

 

        (1)  Mouth and Oral Ulcers --- An Open sore caused by a break in the mucus membrane lining the mouth and may take the form of round or oval, shallow, slight, gray, or yellow spots with an inflamed red border occurring singly on in clusters anywhere in the mouth or oral cavity.  These may also take the appearance as aphthous ulcers or a close resemblance to that of the Herpes Simplex or cold sore.

 

        (2)  Peptic Ulcers --- A raw area that occurs in the gastrointestinal tract where it is bathed by acid gastric juice and usually occurs in the esophagus, stomach, or duodenum and may be singly or in several places with burning or gnawing pain in the abdomen of varying degrees of intensity, belching, bloated feeling, weight loss, nausea, and vomiting and on occasions bleeding with further intensified pain and evidences showing as red blood in the vomit and black streaks or spots in the faeces.  Chronic blood loss thru that means may result in iron-deficiency anaemia.  Intestinal perforation, an ulcerated hole, may cause severe inflammation and peritonitis in the abdominal cavity. 

 

       (3)  Ulcerated Colitis --- Chronic inflammation and ulceration of the lining of the colon and rectum and usually appears as bloody diarrhoea with faeces also containing pus and mucus accompanied by abdominal pain, tenderness, fever, and general malaise, and resulting anaemia from the blood loss.   Other symptoms include rashes, arthritis, conjunctivitis or uveitis. 

 

(E)  Optical and Visual Disturbances --- The eyes may be affected in several different conditions such as Retinal Vasculitis, Iritis and Uveitis, Optic atrophy and Trichiasis. 

 

      (1)  Retinal Vasculitis --- Inflammation of the blood vessels in the eyes which causes visual disturbances which may or may not lead to permanent impairment. 

 

      (2)  Iritis --- Inflammation of the iris within the eye. 

 

      (3)  Uveitis --- Inflammation of the uvea, which may include the iris (Ititis), the ciliary body (Cyclitis), or the choroid (Choroiditis) and all of which may seriously affect vision. 

 

     (4)  Opticatrophy --- The shrinking or wasting of the optic nerve fibers, which results in some or near total loss of vision.

 

     (5)  Keratoconjunctivitis ---  Excessively dry eyes which is commonly seen in Sjogren's Syndrome, and which causes itching and burning of the eyes and creating the sensation of a foreign body under the eyelid. 

 

     (6)  Trichiasis or commonly called "Inverted Eyelashes" --- A condition in which the eyelashes turn or grow inward toward the eyeball and may rub against the cornea causing corneal abrasion and jelly-like filled blisters in the corners of the eyes and occasionally extending to cover the entire white area in severe cases thus giving a very irritated and burning sensation in the eyes. 

 

(F)  Genital Ulceration --- Appearing as Vaginal and Cervical Ulcerations, Epididymitis or Orchitis, Hematuria or Glomerulonephritis or Protenuria.

 

     (1)  Vaginal and Cervical Ulcerations ---Painful, sore, and sometimes fluid seeping skin or mucous membrane eruptions much like that of oral ulcerations occurring both externally on and around the vulva of a woman and internally on the vaginal walls and cervix and may present with appearance similar to that of Herpes Simplex Virus.

 

     (2)  Epididymitis or Orchitis --- Inflammation of the epididymis with fluid-filled cysts-like swellings appearing on the scrotum and penis, and, inflammation of the orchitis or testicles (a long coiled tube-connecting the vasa efferentia, which is the small tubes leading from the testicles, to the vas deferens which is the sperm duct leading to the urethra. 

 

     (3)  Cystitis or Hematuria --- Cystitis is Inflammation of the inner lining of the  bladder usually noted by intense burning and pain upon urination and a constant feeling of urgency to urinate.   Hematuria is the red blood cells in the urine which may be readily visible or small amounts may give a smoky appearance and may enter the urine at any point along the urinary tract from the kidney to the urethral opening.

 

     (4)  Proteinuria and Glomerulonephritis --- Proteinuria is the passage of increased protein in the urine which usually results from damage to the glomeruli or filtering units in the kidneys.   Glomerulonephritis is inflammation and damage to the glomeruli or filtering units of the kidneys that results in increased amounts of proteins and certain other body minerals such as potassium and calcium being excreted from the body in the urine and often resulting in deficiencies. 

 

(G)  Fatigue --- The constant feeling of extreme exhaustion and inability to perform duties and tasks even after periods of resting both short and long and accompanied by malaise, a vague feeling of being sick or of physical discomfort and the need or desire to sleep. 

 

(H)  Auditory Disturbances --- Affecting the ears with vertigo and Tinnitus resulting in hearing loss. 

 

      (1)  Vertigo --- The illusion that one's surroundings or self are spinning either horizontally or vertically and is usually the result from a disturbance of the semicular canals in the inner ear or the nerve tracts leading from them. 

 

     (2)  Tinnitus --- A Ringing, buzzing, whistling, hissing, or other noise heard in the ear in the absence of environmental noise and is almost always associated with hearing loss. 

 

(I)  Bronchial Disturbances --- Which include asthma-like symptoms of Bronchospasms, hemoptysis, embolus, pleurisy, and shadows on chest x-rays. 

 

     (1)  Bronchospasms --- Mimicking asthma and allergic reactions of the lungs and seen as temporary narrowing of the bronchi (airways into the lungs) caused by contractions of the muscles in the lung walls, by inflammation of the lung linings or by a combination of both, which may also cause the release of substances during allergic reactions. Other causes of these brochospasms may include respiratory infection, chronic lung disease (emphysema and bronchitis), or an allergic reaction to chemicals. 

 

     (2)  Hemoptysis --- The medical term for coughing up blood usually as a result of inflammation

 

     (3)  Embolus --- Which is a clump of material that is present in the blood circulation, where is travels eventually to cause arterial obstruction. 

 

     (4)  Pleurisy --- Inflammation on the pleura (membrane linings of the lungs and chest cavity) and usually the result of a lung infection which causes sharp chest pain that sometimes travels to the tip of the shoulder on the involved side and is worse when breathing in and arises because the two inflamed membranes rub across each other.

 

     (5)  Shadows on Chest x-rays appear as darkened areas usually indicating inflammation on or within lung tissues. 

 

(J)  Vasculitis --- Inflammations of the blood vessels and arteries and may occur anywhere in the body the blood travels and usually leads to damage of the linings of those vessels and arteries with narrowing and restriction or blockage of the blood flow resulting in tissue destruction and damage in those areas affected by these vessels and arteries.   Immune complexes consist of antigens (foreign materials such as components of micro organisms) bound to antibodies that have been formed in response to the antigens and are normally destroyed by phagotes (types of white blood cells), but sometimes settle in the walls of the blood vessels, where they cause severe inflammation. 

 

     (1)  Thromboplebitis --- Inflammation of part of a vein, usually near the surface of the body along with a clot formation in the affected segment and can occur as a result from a minor injury to a vein or develop as a complication of Varicose Veins or other blood vessel disorders such as Buerger's Disease and characterized by obvious swelling and redness along the affected segment of the vein and most often accompanied by fever and malaise and is tender to the touch. 

 

          (a)   Varicose Veins --- Twisted and distended veins beneath the skin and most frequently seen in the legs, although they can occur other locations through-out the body. 

 

           (b)  Berger’s Disease, also called Thromboangiitis Obliterans ---

 

                 In which the arteries, nerves, and veins in the legs and occasionally the arms become severely inflamed thus narrowing the arteries blocking off blood supply to the toes and fingers eventually causing gangrene.   The main symptom is pain in the hands and feet with victims suffering cold sensitivity and appearance of hands turning white, then blue, then red in cold conditions and which is also called Raynauds’s Disease. 

 

   (2)  Deep Vein Thrombosis --- The clotting of blood within deep-lying veins, usually in the legs and thought to most often be the result of sluggish blood flow when a person lies or sits still for long periods of time.  Clots in these veins may cause symptoms such as pain, tenderness, swelling, discoloration, and ulceration of the skin. 

 

   (3)  Aneurism --- Ballooning to an artery due to the pressure of blood flowing through a weakened area, frequently the result of inflammation in the surrounding artery area and often threatens and/or do rupture with very serious and severe consequences and tissue damages. 

 

(K)  Prickly Reaction Skin Test --- Whereby pricking the skin with a sterile needle or injecting a sterile saline solution under the skin will develop a small papule or pustule at the site of the prick or injection.   This test is not always positive even when Behcets Disease and inflammation are active, but may be considered a positive sign or symptom when a positive reaction does occur.