The most common form of Raynaud's Disease is ideopathic primary Raynaud's Disease and is found in young women in 60-90% of the cases reported. Secondary Raynaud's Disease may be due to other connective or soft tissue disorders such as rheumatoid arthritis, systemic lupus or scleroderma. Occasionally, secondary Raynaud's Disease may be due to peripheral vascular occlusive disease, neurogenic lesions or drug intoxications.

The cause of Raynaud's Disease is unknown.  Any condition or contributing factor that results in peripheral vasospasm may be considered a contributing cause of Raynaud's Phenomenon and Raynaud's Disease.  Contributing conditions include anxiety, depression, rheumatoid arthritis, systemic lupus (SLE) and scleroderma.  Contributing factors include cold exposure and nicotine.  Raynaud's Disease is often seasonal and most active in the cold weather months.  Exposure to cold air (air conditioning) during the summer can also initiate the onset of Raynaud's Phenomenon. 

The picture to the left shows an interesting case of Raynaud's Disease that effects only the weight bearing surface of the digits.  This 73 y/o patient described a 3 year history of sores on the bottom of her toes that began during the winter months and ended in spring with the onset of warm weather.  The patient was not active and sat for long periods of time with her feet flat on the floor.  The ischemic changes seen in the skin are specific to the weight bearing portions of her toes.  This was caused by a combination of arterial vasospasm (Raynaud's Disease) and direct pressure to the skin by the floor that inhibited normal blood flow to the plantar surface of the digit.  Treatment for this problem included linitation of cold exposure and elevating the feet to eliminate floor pressure against the toes. 

Treatment of Raynaud's Phenomenon and Disease

Initial treatment consists of avoidance of any contributing factors to the condition such as cold exposure, emotional swings or smoking. Nicotine is a potent vasoconstrictor. Sedatives, mood stabilizers and psychological counseling may be helpful to control contributing factors such as stress and anxiety.

Visual imagery techniques are helpful for some patients. As an example;

Wendy has had problems with Raynaud's Disease for years. She is a mom and employed as a banker. On days where she is particularly busy, her fingers will blanch white and become cold. Wendy has found that she can control these symptoms by stopping everything that she is doing. She folds her hands in her lap and visualizes in her mind a beam of sunlight coming through a window focused on her hands. She feels the warmth of the sun on her hands. Wendy performs this exercise as needed. In addition to improving the temperature of her hands, she also notices a reduction in her stress level and tightness in her back.

Topical L-arginine cream can help warm hands and toes. L-arginine cream is a good prophylactic trick to pre-empt cold exposure and is handy for going outdoors in cold climates in winter. In-shoe warming devices are also helpful in this regard. Several oral medications are helpful in reducing peripheral vasospasm. Those medications include calcium channel blockers such as nifedipine (Calan/Verapamil) and prazosin. A surgical procedure performed by a neurosurgeon, called a sympathectomy, is reserved for the most severe cases.

Arterial foot and leg ulcers are usually caused by arteriosclerosis obliterans (ASO), a progressive occlusive disease of the medium to large arteries. ASO is believed to begin by the formation of a fatty streak in the artery called atherosclerosis. These fatty streaks localize in the wall of the blood vessel and harden over time forming ASO. Complete occlusion of the artery results in disruption of blood flow to the target organ. In the case of a coronary artery, the end target organ is the heart and the result of arterial occlusion is a heart attack.

Arterial foot and leg ulcers occur in the same manner. Occlusion of the arteries of the leg impair healing resulting in ulcerations. Males are more predisposed to ASO. Other contributing factors include mechanical stress, lipid disorders and high blood pressure. Tobacco use, whether smoking, snuff or chew is a significant contributing factor. Circulating levels of nicotine make the blood more acidic. As a result, nicotine makes the artery wall much more permeable to atherosclerotic plaque, increasing the likelihood of ASO.

Wagner described the classification used to describe arterial ulcers and wounds used most commonly today.

Wagner Grade 0 - Irritation of the skin with no break in the skin.

Wagner Grade 1 - Superficial wound with no infection. Skin is intact but erythematous.

Wagner Grade 2 - Partial to full thickness erosion of the skin. Infection possible. No deep tissue or bone involvement.

Wagner Grade 3 - Full thickness, deep tissue involvement. Wound is infected with probably bone infection.

Wagner Grade 4 - Deep, extensive infection with gangrene.

 

Treatment of Ischemic Arterial Foot and Leg Ulcers

The underlying cause of arterial foot and leg ulcerations is ischemia (lack of blood flow). Effective treatment of ischemia may include many different methods of care. Patients will benefit greatly from smoking cessation. Increased exercise may contribute to collateral circulation and may improve blood flow to the ulcer.

Several medications are used to improve arterial blood flow. Calcium channel blockers (Verapamil, Calan), although primarily used as antihypertensive agensts, may be used to increase small vessel peripheral blood flow. Trental (pentoxifylline) is an oral medication that is used to decrease the viscosity of the blood. Trental coats the red blood cell so that it becomes slippery and able to travel through occluded vessels. Pletal became available in '00. Pletal (cilostazol) inhibits cellular phosphodiesterase resulting in dilation of arterial vascular beds. Recent studies have shown a significant reduction in intermittent claudication symptoms with Pletal.

Surgical revascularization is a common procedure but may only be used on the medium to large vessels of the leg. Until recently, revascularization was limited to bypass grafting with harvested grafts or synthetic grafts. During the past ten years, advances in endovascular techniques has made revascularization much more successful. Endovascular techniques focus on cleaning the blockage of the leg by using balloons to compress plaque or devices to ream plaque, re-opening occluded vessels.

Wound care is also important for arterial ulcerations. Treatment of infection any be necessary with antibiotics, whether topical, oral or IV. Protection of the wound is essential. Many devices have been invented for this purpose to act as a cradle or pad for the wound. Many physicians use topical enzymes for wound debridement or wound growth factors.

The reason some children are born with clubfeet is not clearly understood. Several authors have speculated that the deformity stems from an under developed bone in the foot called the talus. As the talus grows in the young fetus, the bone 'unfolds' from an inverted (varus) position. This unfolding process seems to occurs by the neck of the talus straightening over the first several months of fetal growth. Any disruption of the straightening process may contribute to a delay or arrest of the straightening resulting in a residual inverted (varus) position of the foot. Some authors have speculated that this delay or arrest may be due to a decrease or interruption in the blood flow to the neck of the talus.

X-rays taken of the infant clubfoot will show inversion of the talus in relationship to the calcaneus. This classic x-ray view used to evaluate clubfeet is referred to as Kite's Angle. X-ray evaluation of clubfeet includes a comparison of the deformity in relationship to the bones of the lower leg (ankle), the bones of the rearfoot (talus and calcaneus) and the relationship of the rearfoot bones to the forefoot.

Most individuals in western countries who are born with clubfeet will mature to have full and productive lives. And adult who was treated as a child for clubfeet will show characteristic findings of the lower extremity.  Some of these characteristic findings includes a thin calf, called a stork deformity. A characteristic 'C' shaped foot is also common as a result of under treated metatarsus adductus.

Unfortunately, many cases of untreated clubfeet in adults may be seen in under developed countries through-out the world. These patients walk on the side of their foot and may be extremely limited in the amount of time that they can stand.

Treatment of clubfoot

The foot of a newborn is merely the size of an adult thumb. As the foot matures, the development of the bones and joints become rigid and less flexible. Therefore, the earlier treatment is initiated, the better the potential for a good outcome. Treatment may be conservative, surgical or a combination of both. The decision as to which method of treatment depends in a great majority of cases on the degree of deformity at the talar neck (see the anatomy tab for further information regarding the talus).

Manipulation and casting are commonly used as conservative measures in treating neonatal clubfoot. Parents are instructed by their physician in techniques that will help to correct the contraction of the posterior and medial ankle and foot. Manipulation may be reinforced by the use of casts or braces. Several new removable braces have been developed in the past decade that have virtually eliminated the need for plaster or fiberglass casting (for additional information see The Wheaton Brace Company). It is not unusual for the clubfoot deformity to be corrected within the first 2-3 months of life. Most importantly, the correction must be maintained with splints, braces and corrective shoes.

Should three or four months of stretching, manipulation and casting not reduce the deformities of a clubfoot, surgical correction may be indicated. The most frequent deformity left following a period of conservative care is the rearfoot deformity of inversion (varus) and plantarflexion (equinus). Metatarsus adductus, on the other hand, seems to be much more easily reduced by conservative care. Surgical release of the posterior and medial compartments will usually correct the residual rearfoot deformities.

After the age of two, the deformities of a clubfoot become much more difficult to manage due to thickening and fibrosis of the soft tissue structures of the posterior and medial ankle and foot. Most of the procedures used to treat clubfoot in the child older than 4 years include some modification of the bony structures of the foot.

Although most clubfeet in developed countries are addressed and treated when the patient is an infant, many residual deformities of clubfeet carry over into the adult foot. Residual metatarsus adductus, calcaneal varus and inversion of the foot are common. Clubfoot deformity also leads to early degenerative arthritis of the foot.

Onychomycosis is a fungal infection that is unrelated to foot hygiene. No matter how clean you keep your feet, you are still at risk for a fungal infection of the skin and nail. There are several ways in which we contribute to the onset of onychomycosis. First, as a civilized society we wear shoes. Shoes create an environment that is wonderfully conducive to the growth of fungus. The environment inside a shoe is dark, warm and damp. That environment is perfect for the growth of fungus. Second, any form of trauma to the nail will enable fungus to enter the space beneath the nail and begin an infection.

It is safe to say that 50% of folks over the age of 50 show clinical signs of onychomycosis. But this doesn't necessarily mean that onychomycosis is due to 'old age'. This simply implies that with the passage of time there is a greater tendency to acquire a fungal infection of the nail. Trauma makes the nail much more susceptible to fungal infections. An injury to the nail is a common precursor to a fungal nail infection. It's also reasonable to assume that folks in professions that abuse their feet would tend to have a higher rate of onychomycosis. Trauma may be something abrupt such as a can of soup hitting the nail from the top shelf or something as benign as a pair of ill-fitting shoes constantly rubbing on the nail. Onychomycosis is also very common in runners.

The appearance of onychomycosis can vary but most cases begin at the distal tip of the nail and slowly progress into the nail over a period of months to years. This classic onset of onychomycosis is called distal subungual onychomycosis. The nail will thicken as the fungus continues to grow. The filaments of fungus take up space in the nail causing it to swell. The nail can be yellow, white or even green to black. The nail also begins to be chalky, flakey and will separate from the underlying nail bed.

Treatment of toe nail fungus and onychomycosis

Prevention of injuries to the nail is a very important aspect of preventing fungal nail infections. Preventative measures include:

• Avoid injuries to the nails. Protect the feet with enclosed shoes or steel toe boots.

• Keep the feet dry with frequent changes of socks and use of talc or baby powder. Rotate shoes to let them dry for 48 hours between use.

• Purchase shoes with a toe box the shape of your foot. When purchasing shoe, stand barefoot next to the new shoes and see how the shape of your foot compares to the toe box of the shoe. Try to match the shape of the toe box to the shape of your foot.

Medications for onychomycosis fall into two categories; topical and oral. There are any number of effective topical antifungal medications available over the counter. Topical medications are most helpful in treating early infections and for maintaining clear nails. Topical medications are fungistatic meaning that they inhibit the growth of the fungus. By limiting the growth rate of the fungus, the nail is then able to grow faster than the invading fungus infection. Remember, fungus doesn't take a day off. Compliance is a big issue when using topical antifungals. It can often take several months before results are seen.

The older generation of oral antifungal medications, which includes medications such as Fulvicin or Griseofulvin, have been used successfully for years and are making a comeback due to their economic value. The newer generation of oral antifungals, including Sporanox and Lamisil, have been received very well by the medical community. These medications should only be taken under the care of your doctor due to potential hepatic toxicity.

It's important to note that one of the characteristic findings of fungal nail infections is the separation of the nail from the underlying nail bed. Topical and oral medications are in part limited in their efficacy in that they can treat the fungal infection of the nail but they are not able to reattach the nail to the nail bed. Separation of the nail from the nail bed occurs in advanced cases of onychomycosis. Therefore, the success or failure of these medications in their ability to return the appearance of the nail to 'normal', can be limited by how advanced the nail infection is at the onset of treatment.

When all else fails, the fungal toe nail can be permanently removed. This procedure is an office based procedure and most patients return to their normal shoes in just a Band-Aid within 24hrs. The removal of the fungal to nail is permanent.

Which treatment choice is right for you? Consider the following two examples;

Case 1. Sandy is a 24 year old hairdresser who has intimate hands on contact with her clients on a daily basis. She has developed a fungal infection in several of her finger nails. She is concerned that the fungal infection will have a direct impact on her livelihood and does not want to spread the infection to others. In this case, Sandy's fungal infection may directly affect her job. In this case, the use of an oral antifungal may be indicated.

Case 2. Joe is a 62 year old farmer and has a long history of injuries to his hands and feet. His last visit to the doctor showed signs of an increase in his liver enzyme studies indicating an overall decrease in his liver function. Joe has developed onychomycosis in most of his toe nails. Joe would not be a candidate for oral antifungal medications. In fact, Joe may not be a candidate for treatment at all. Periodic debridement of the nail may be all that is necessary.

Case 3. Irene is a 42 y/o single mom who works in an office setting. Recently she has noticed a yellow discoloration of several of her toenails. Irene is beginning to see the onset of onychomycosis. Irene would be a great candidate for topical antifungal nail medications.

reflex sympathetic dystrophy syndrome (RSD/RSDS)
Sudeck's atrophy
shoulder-hand syndrome
algodystrophy
peripheral trophoneurosis
sympathetically maintained pain
post-traumatic pain syndrome
sympathalgia
sympathetic overdrive syndrome

Due to confusion arising from the many names for this set of symptoms, The International Association for the Study of Pain (IASP) developed nomenclature to more accurately describe CRPS.

CRPS I - Consists of pain, sensory abnormalities, abnormal sweating and blood flow, abnormal motor system function and trophic changes ( thickening of the skin and nails, coarse thin hair growth) and atrophy of the superficial and deep tissues (skin, muscle, bone). The most common form is RSD and may not present with an identifiable nerve injury.

CRPS II - Same as CRPS I but presents with an identifiable nerve injury. Symptoms include burning pain made worse by light touch, temperature changes or motion of the limb. These findings are most common in the foot or hand following partial injury to the nerve. The affected area appears cool, reddish, and clammy. The superficial and deep tissue structures may also begin trophic changes.

 

Treatment for complex regional pain syndromes

Treatment consists of many different measure, but there is general agreement that the success of treatment depends upon early implementation of treatment. Treatment may include;

Medications
Narcotics- for pain suppression
Anti-inflammatory- non-narcotic control of inflammatory pain
Antidepressants-maintenance of normal sleep cycles, anxiety control
Calcium channel blockers- increased blood flow to extremities
Anticonvulsant- regulation of normal sleep cycle, control of pain

Pain blocks
Peripheral nerve blocks of the affected area
IV regional blocks of the affected extremity
Lumbar sympathetic blocks- given by an anesthesiologist

Physical therapy
Range of motion, strengthening exercises, continuous passive motion
(CPM)
Whirlpool, ultrasound, heat treatment
TENS, nerve stimulation


Steroid injections

Lumbar sympathectomy

Dorsal column stimulation
Morphine pump

Neurectomy - surgical excision of the nerve

The prognosis for patients with CRPS varies greatly and depends upon the degree of symptoms, when treatment is initiated and the type of treatment. Studies have shown that the overall success rate of the treatment of RSD has been 50%. In a study performed by Anderson and Fallat, they found that 3.5 years following the onset of traumatic injury resulting in CRPS, 12 of 13 patients still had pain considered to be moderate to considerable. (1)

Another aspect of CRPS treatment is ongoing psychological counseling.  CRPS I and II have a significant bearing on the psychological well being of the patient.  A feeling of hopelessness, anger and frustration only helps to perpetuate CRPS.  Psychological counseling enables CRPS patients to take control of the course of their problem.  Concurring the feeling of helplessness is actually a very important aspect of treating CRPS.