Lichen Myxedematosus

The terms lichen myxedematosus, papular mucinosis, and scleromyxedema are used to describe different forms of the same disorder, a chronic connective tissue disorder in which mucin deposits form in the skin. Lichen myxedematosus or papular mucinosis refers to the less severe form that is limited to the skin. Scleromyxedema refers to the rare, more severe form in which other body systems are affected in addition to the skin.

The cause of lichen myxedematosus and scleromyxedema is not known. The diseases affect both males and females and individuals of all ethnic backgrounds. Symptoms usually begin in adulthood.

Symptoms
Lichen myxedematosus or papular mucinosis – Bumps (papules), either skin-colored or reddened, appear on the skin, and may form groups. The papules may have a pattern of parallel ridges. Papules are found on the backs of the hands, on the face, or the arms and legs. The ridges and folds of the face may be distorted, with ridges and furrows developing on the brow and cheeks. This is known as leonine (“lion-like”) facies (leontiasis).
Scleromyxedema – Areas of red, raised skin appear, and the skin becomes tight like in scleroderma. There is difficulty moving the face, fingers, and arms and legs. Scleromyxedema also causes problems with the lungs and breathing, and with the heart and circulation. It may cause muscle weakness and joint pain, and neurological symptoms like those in brain disease, such as seizures, may be present.

Diagnosis
Diagnosis of lichen myxedematosus and scleromyxedema is based on the symptoms the individual is experiencing, especially what the skin looks like. A special blood test for a paraprotein (7S-IgG) can be done, and is usually positive. Other laboratory tests, including thyroid function tests, are normal. Skin and tissue samples can be examined under a microscope for deposits of mucin, which are characteristic of the disease.

Treatment
Many types of treatment for lichen myxedematosus and scleromyxedema have been tried in small numbers of people in research studies, but none has been consistently effective in reducing symptoms. Symptoms may improve with the use of high-dose dexamethasone and/or thalidomide, but over time the symptoms may return or even worsen. Chemotherapy drugs such as melphalan have also been tried, but without consistent success in treatment.

Individuals with lichen myxedematosus and scleromyxedema have long-term, often disfiguring, and possibly disabling disorders. The risks and benefits of drug treatment should be carefully considered.

Research
Researchers at the University of Texas M.D. Anderson Cancer Center studied eight individuals with scleromyxedema who had the disease for 3-1/2 years and had other symptoms in addition to the skin symptoms. All the individuals underwent high dose chemotherapy with melphalan followed by autologous stem cell transplantation. Seven of the eight individuals had a marked improvement in their symptoms after the transplant. In five individuals, the skin symptoms resolved completely. The researchers concluded that high-dose melphalan appears to be a safe and effective treatment, but also recommended further studies be done. The research was published in the January 15, 2006, issue of Blood.

Information for this article was taken from:
- Donato, M.L., et al. Scleromyxedema: Role of high-dose melphalan with autologous stem cell transplantation. Blood, 107; 415, pp. 463-466
- Liotta, E. A. (2005). Lichen myxedematosus. eMedicine, accessed at http://www.emedicine.com/derm/topic231.htm
- Scleromyxedema Survivors

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Stem Cell Transplant May Reverse Type 1 Diabetes

Type 1 diabetes is a lifelong disease that affects more than a million American children and adults. It occurs when the person’s own immune system attacks and destroys the insulin-producing beta cells in the pancreas. Since the cells no longer produce insulin, people with type 1 diabetes must supply it themselves. This means undergoing multiple injections throughout the day and night, or having insulin delivered by an insulin pump through a permanent tube under the skin. But scientists may have found a way to free type 1 diabetics from their constant struggle.

In a dynamic new study, Dr. Julio Voltarelli of the University of São Paulo in Brazil and colleagues from Northwestern University in Chicago were able to reverse the course of type 1 diabetes with a patient’s own stem cells. “I wouldn’t use the word cure,” said Dr. Richard Burt, one of the co-authors from Northwestern. “But it appears we changed the natural history of the disease. It’s the first therapy for patients that leaves them treatment-free—no insulin, no immune suppression for almost five years.”

Burt reported the team’s initial achievement in 2007, with 15 diabetic patients who received their own stem cells and no longer needed insulin to control their blood sugar levels. In this follow-up, they detail the same success with eight additional patients. Insulin-free time lasted more than four years for one patient, more than three years for four patients, more than two years for three patients, and at least one year for four patients. Eight had to go back to using insulin, but required lower doses than they did before the procedure.

To confirm the lasting effects of the therapy, the researchers measured the patient’s levels of C-peptides, which show how well the body is producing insulin. They found those levels increased in 20 of the 23 patients. And even the eight who relapsed still had significantly higher levels of C-peptide by 36 months. “Because late complications of diabetes on the microvascular compartment are inversely related to C-peptide levels, it is probable that even those patients who resumed insulin are at lower risk for long-term diabetes complications,” the researchers said.

The strategy behind the procedure, known as autologous non-myeloablative hematopoietic stem-cell transplantation, is simple. First, CD34 stem cell, which can grow into all white blood cells of the immune system, are extracted from the patient’s bone morrow and stored. Next, the patients receive drugs that destroy what remains of their immune system, at the same time eliminating the components of the immune system that attack the islets. Finally, the stored stem cells are returned to the patient and left to regenerate a fresh immune system that will no longer attack the islet cells.

Burt says a larger randomized trial is now planned in both Brazil and in the United States. As with the existing trial, newly-diagnosed patients will be selected but this time, some of the patients will receive placebo. Other researchers are pursuing new stem cell options, including stem cells that can be grown from a patient’s own skin, which would eliminate the need for extracting immune stem cells from bone marrow. “Every door that we open leads to another door,” said Burt. “All research is built by sitting on the shoulders of other studies. This trial is something that will contribute to and move the field of stem cell therapy forward.”

A similar procedure developed by Burt has already shown success in another autoimmune disease—multiple sclerosis. Results published in January showed that many patients benefitted through the reprogramming of their immune systems, this time to stop it from attacking nerve and brain cells.

Living with diabetes is a daily challenge. In addition to having to check their insulin levels and take injections, diabetics must also carefully balance their food intake and their exercise to regulate their blood sugar levels in an attempt to avoid hypoglycemic (low blood sugar) and hyperglycemic (high blood sugar) reactions, which can be life-threatening. Diabetes is associated with long-term complications that affect almost every part of the body. The disease often leads to blindness, heart and blood vessel disease, stroke, kidney failure, amputations, and nerve damage.

In recent years, pancreas transplants have been used to treat type 1 diabetes, but not only is the operation itself serious, the side effects of the drugs required to prevent the immune system from fighting the new organ may be worse than the problems caused by diabetes. Islet cell transplant is also being investigated and has been achieved in rodents and in experimental trials in humans. However, the function of transplanted islets is lost over time, and few recipients are able to stop using insulin for very long. They also have side effects from immunosuppressive drugs.

The new findings were reported in the April 15 issue of the Journal of the American Medical Association.

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New Prostate Cancer Vaccine Shows Promise

There are many health concerns today when it comes to illnesses, but one of the number one health concerns for a man to consider is prostate cancer. There are already treatments available for this type of cancer, but now there is a new one to consider. Provenge, which an experimental treatment vaccine for advanced prostate cancer, has met researchers’ goal in a key trial that is needed to get FDA approval. This news was recently announced by Dendreon, which is the company that makes the vaccine Provenge.

Mitchell Gold, M.D. and the president and chief executive of Dendreon, said that they believe that this vaccine is truly a breakthrough for the prostate cancer community a testament to the promise of the field for cancer immunotherapies. Provenge is a biologic drug that is given by infusion to spur the immune system to help fight against advanced prostate cancer that does not respond to anti-androgen treatment.

In 2007, an FDA advisory panel recommended that the FDA approve the new vaccine Provenge. However, the FDA requested that more information be collected to see whether Provenge prolongs survival. This request led to a new study of approximately 512 men that has advanced prostate cancer. These men had metastatic, androgen-independent prostate cancer, which means their cancer had spread and it was not responding to the anti-androgen treatment.

In this study, the overall survival was significantly better for the men that were taking Provenge than those that were taking a placebo. The results of this study were “unambiguous” and “very consistent” with previous trials for Provenge, says Gold. Dendreon plans to submit the results of this study to the FDA in the fourth quarter of 2009, and after that the FDA will have approximately six months to review all of the material.

Gold stated that this new data can support the use of Provenge being used as a frontline treatment in men that have metastatic, androgen-independent prostate cancer. He also noted that no new side effects from the vaccine stood out in the recent study. In the previous trials conducted, the most common side effects in the men taking Provenge were headache, chills, shortness of breath, fever, tremor, fatigue, and vomiting, mainly at a low level and for one to two days following the infusion of the vaccine.

Gold said that most of the men would first have surgery or some other form of local therapy, then the anti-androgen therapy if their cancer recurred, and if their PSA levels rose after these steps, then the Provenge vaccine would come into play as potential treatment option for them if these others didn’t work. In the men that had prostate cancer, PSA or prostate-specific androgen levels are used to help gauge the success of the treatment for the cancer.

However, at this time Dendreon is not releasing any further details of this study until April 28th, when the findings of the study will be presented at the American Urological Association’s annual meeting held in Chicago. The technology that is used to make the Provenge vaccine may also prove to be useful against other forms of cancer as well, Gold says.

The American Cancer Society has also released a statement about the Provenge news. The statement was made by Otis W. Brawley, MD, and the chief medical officer at the American Cancer Society. Brawley stated that Dendreon’s announcement about the new study for vaccine Provenge has shown reason for optimism for a vaccine that has generated controversy for the past several years. “We have to respect the scientific process, an important part of which is a full disclosure and careful review and discussion of the data, which the company says will not be released until an upcoming medical meeting. One of the most important question’s we’ll be looking at will be the magnitude of the survival advantage; how much longer the men taking the vaccine lived compared to those on standard therapy. As with any new therapy, it will take a detailed analysis to fully understand the impact of this potential new treatment for patients with advanced prostate cancer. We look forward to the presentation of the study at the upcoming meeting.”

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Ulnar nerve dysfunction

Definition

Ulnar nerve dysfunction is a problem with the nerve that travels from the wrist to the shoulder, which leads to movement or sensation problems in the wrist and hand.

Alternative Names

Neuropathy - ulnar nerve; Ulnar nerve palsy

Causes

Ulnar nerve dysfunction is a common form of peripheral neuropathy. It occurs when there is damage to the ulnar nerve, which travels down the arm. The ulnar nerve is near the surface of the body where it crosses the elbow, so long-term pressure on the elbow may cause damage.

The damage involves the destruction of the nerve covering (myelin sheath) or part of the nerve (axon). This damage slows or prevents nerve signaling.

A problem with one single nerve group (such as the ulnar nerve) is called mononeuropathy. The usual causes are:

• Direct injury
• Long-term pressure on the nerve
• Pressure on the nerve caused by swelling or injury of nearby body structures

Entrapment involves pressure on the nerve where it passes through a narrow structure.

The ulnar nerve is commonly injured at the elbow because of elbow fracture or dislocation. Prolonged pressure on the base of the palm may also damage part of the ulnar nerve. Temporary pain and tingling of this nerve is common if the elbow is hit, producing the experience of hitting the "funny bone" at the elbow.

In some cases, no cause can be found.

Symptoms

• Abnormal sensations in the 4th or 5th fingers, usually on the palm side
• Numbness, decreased sensation
• Pain
• Tingling, burning sensation
• Weakness of the hand

Pain or numbness may awaken you from sleep. Activities such as tennis or golf make the condition worse.

Exams and Tests

An exam of the hand and wrist can reveal ulnar nerve dysfunction. Signs may include:

• "Claw-like" deformity (in severe cases)
• Difficulty moving the fingers
• Wasting of the hand muscles (in severe cases)
• Weakness of wrist and hand bending

A detailed history may be needed to determine the cause of the neuropathy.

Tests may include:

• Blood tests
• Imaging scans
• Nerve conduction tests
• Recording of the electrical activity in muscles (EMG)
• X-rays

Treatment

The goal of treatment is to allow you to use the hand and arm as much as possible. The cause should be identified and treated. Sometimes, no treatment is required and you will get better on your own.

Treatments may include:

• A supportive splint or elbow pad to help prevent further injury
• Corticosteroids injected into the area to reduce swelling and pressure on the nerve
• Surgery to relieve pressure on the nerve, if the symptoms get worse, movement is difficult, or there is proof that part of the nerve is wasting away. Surgical decompression may be recommended if the symptoms are from entrapment of the nerve.
• Over-the-counter analgesics or prescription pain medications to control pain (neuralgia)
• Other medications, including gabapentin, phenytoin, carbamazepine, or tricyclic antidepressants such as amitriptyline or duloxitine, to reduce stabbing pains.
• Physical therapy exercises to help maintain muscle strength
• Occupational counseling, occupational therapy, job changes, or retraining

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Bakers cyst

Definition

Baker's cyst is an accumulation of joint fluid (synovial fluid) that forms behind the knee.

Alternative Names

Popliteal cyst

Causes

A Baker's cyst may form by the connection of a normal bursa (a normal lubricating fluid sac) with the knee joint. This type is more common in children.

The condition can also be caused by the herniation of the knee joint capsule out into the back of the knee, which is more common in adults. This commonly occurs with a tear in the meniscal cartilage of the knee.

In older adults, Baker's cysts are frequently associated with knee arthritis.

Symptoms

A large cyst may cause some discomfort or stiffness but generally has no symptoms. There may be a painless or painful swelling behind the knee.

The cyst may feel like a water-filled balloon. Occasionally, the cyst may rupture, causing pain, swelling, and bruising on the back of the knee and calf

It is very important to tell the difference between a ruptured Baker's cyst and a blood clot (deep venous thrombosis), which can also cause pain, swelling, and bruising on the back of the knee and calf. A blood clot may be dangerous and requires immediate medical attention.

Exams and Tests

During a physical exam, the doctor will look for a soft mass in the back of the knee. If the cyst is small, comparing the affected knee to the normal knee can be helpful. There may be limitation in range of motion caused by pain or by the size of the cyst. In some cases there will be signs and symptoms of a meniscal tear.

Transillumination, or shining a light through the cyst, can demonstrate that the mass is fluid filled.

If the mass demonstrates any abnormal signs, like rapid growth, night pain, severe pain, or fever, a more involved work-up is indicated to rule out non-cystic tumors that can grow in the back of the knee.

X-rays will not show the cyst or a meniscal tear but will show other abnormalities that may be present including arthritis.

MRI can be helpful to visualize the cyst and to demonstrate any meniscal injury.

Treatment

Often no treatment is necessary and the practitioner can observe the cyst over time. If the cyst is painful, treatment is usually aimed at correcting the underlying problem, such as arthritis or a meniscus tear. Removal of the cyst is generally not done because it may damage nearby blood vessels and nerves.

Sometimes, a cyst can be drained (aspirated) or, in rare cases, removed surgically, if the cyst becomes excessively large or causes symptoms.

Outlook (Prognosis)

A Baker's cyst will not cause any long-term harm, but can be annoying and painful. Baker's cysts usually go away on their own, but how fast this occurs varies from person to person.

Long-term disability is very rare, as most cases improve with time or arthroscopic surgery.

Possible Complications

Complications are unusual, but may include:

• Long-term pain and swelling
• Complications from associated injuries, like meniscal tears

When to Contact a Medical Professional

Call for an appointment with your health care provider if there is a swelling behind the knee that becomes large or painful. Pain could be a sign of infection, which is not normally associated with Baker's cyst.

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Cataracts

Cataracts are a common age-related vision problem. About 20.5 million Americans age 40 and older have cataracts, and the older a person gets the greater the risk for developing cataracts. Women are more likely to develop cataracts than men, and African Americans and Hispanic Americans are at particularly high risk.

In addition to age, other factors may increase the risk of cataract development. These include:

• Diabetes
• Smoking
• Over-exposure to sunlight

Symptoms

During the early stages, cataracts may have little effect on vision. Symptoms vary due to the location of the cataract in the eye (nuclear, cortical, or posterior subcapsular). Depending on the type and extent of the cataract, patients may experience the following symptoms:

• Cloudy vision
• Double or blurry vision
• Glare and sensitivity to bright lights
• Colors appear faded
• Difficulty reading due to reduced black-white contrast

Treatment

Cataracts never go away on their own, but some stop progressing after a certain point. But if cataracts continue to grow and progress, they can cause blindness if left untreated. Fortunately, cataracts can usually always be successfully removed with surgery. Millions of cataract surgeries are performed each year in the United States, and there is a very low risk for complications. However, before opting for surgery, patients need to consider on an individual basis how severely a cataract interferes with their quality of life. Cataract surgery is rarely an emergency, so patients have time to consult with their doctors and carefully consider the risks and benefits of surgery.

Cataract Removal Surgery

Surgery involves removing the cataract and replacing the abnormal lens with a permanent implant called an intraocular lens (IOL). The operation takes less than 1 hour and is performed on an outpatient basis. The procedure is generally painless and most patients remain awake during it. If you have cataracts in both eyes, doctors recommend waiting at least 1 month between surgeries.

Introduction

A cataract is an opacity, or clouding, of the lens of the eye.

The lens of an eye is normally clear. If the lens becomes cloudy or is opacified, it is called a cataract.

The prevalence of cataracts increases dramatically with age. It typically occurs in the following way:

• The lens is an elliptical structure that sits behind the pupil and is normally transparent. The function of the lens is to focus light rays into images on the retina (the light-sensitive tissue at the back of the eye).
• In young people, the lens is elastic and changes shape easily, allowing the eyes to focus clearly on both near and distant objects.
• As people reach their mid-40s, biochemical changes occur in the proteins within the lens, causing them to harden and lose elasticity. This causes a number of vision problems. For example, loss of elasticity causes presbyopia, or far-sightedness, requiring reading glasses in almost everyone as they age.
• In some people, the proteins in the lens, notably those called alpha crystallins, may also clump together, forming cloudy (opaque) areas called cataracts. They usually develop slowly over several years and are related to aging. In some cases, depending on the cause of the cataracts, loss of vision progresses rapidly.
• Depending on how dense they are and where they are located, cataracts can block the passage of light through the lens and interfere with the formation of images on the retina, causing vision to become cloudy.

Click the icon to see an image of eye anatomy.

Cataracts can form in any of three parts of the lens and are named by their location.

• Nuclear cataracts. These form in the nucleus (the inner core) of the lens. This is the most common variety of cataract associated with the aging process.
• Cortical cataracts. These form in the cortex (the outer section of the lens).
• Posterior subcapsular cataracts. These form toward the back of a cellophane-like capsule that surrounds the lens. They are more frequent in people with diabetes, who are overweight, or those taking steroids.

Causes

Although older age is the primary risk factor for cataracts, experts are still not certain about the exact biologic mechanisms that tie cataracts to aging.

Oxygen-Free Radicals (Oxidants) and Glutathione

Researchers have been focusing on particles called oxygen-free radicals as a major factor in the development of cataracts. They cause harm in the following way:

• Oxygen-free radicals (also called oxidants) are molecules produced by natural chemical processes in the body. Toxins, smoking, ultraviolet radiation, infections, and many other factors can create reactions that produce excessive amounts of these oxygen-free radicals.
• Oxidants are missing an electron, so they are unstable and tend to chemically bind with other molecules in the body. When oxidants are overproduced, these chemical reactions can be very harmful to nearly any type of cell in the body. At times these reactions can even affect genetic material in cells.
• Cataract formation is one of many destructive changes that can occur with overproduction of oxidants, possibly in concert with deficiencies of an important protective anti-oxidant called glutathione.
• Glutathione occurs in high levels in the eye and helps clean up these free radicals. One theory posits that in the aging eye, barriers develop that prevent glutathione and other protective antioxidants from reaching the nucleus in the lens, thus making if vulnerable to oxidation.

Radiation and Electromagnetic Waves

Sunlight and Ultraviolet Radiation. Sunlight consists of ultraviolet (referred to as UVA or UVB) radiation, which penetrates the layers of the skin. Both have destructive properties that can promote cataracts. The eyes are protected from the sun by eyelids and the structure of the face (overhanging brows, prominent cheekbones, and the nose). Long-term exposure to sunlight, however, can overcome these defenses.

• UVB radiation produces the shorter wavelength, and primarily affects the outer skin layers. It is the primary cause of sunburn. It is also the UV radiation primarily responsible for cataracts. Long-term exposure to even low levels of UVB radiation can eventually cause changes in the lens, including pigment changes, which contribute to cataract development. (UVB also appears to be responsible for macular degeneration, an age-related disorder of the retina.) Some scientists suggest that global warming and ozone depletion may increase peoples exposure to UVB, leading to a greater incidence of cataracts.
• UVA radiation is composed of longer wavelengths. They penetrate more deeply and efficiently into the inner skin layers and are responsible for tanning. The main damaging effect of UVA appears to be the promotion of the release of oxidants.

Radiation Treatments. Cataracts are common side effects of total body radiation treatments, which are administered for certain cancers.

Electromagnetic Waves. Questions have been raised about the hazards of low-level radiation from computer screens. To date, no study has demonstrated an association between cataract development and video display terminals. It is a good idea, in any case, to sit at least a foot away from the front of a screen.

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Juvenile angiofibroma

Definition

Juvenile angiofibroma is a noncancerous growth of the back of the nose or upper throat.

Alternative Names

Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor

Causes

Juvenile angiofibroma is not very common. It is usually found in adolescent boys. The tumor contains many blood vessels, spreads within the area in which it started (locally invasive), and can cause bone damage.

Symptoms

• Difficulty breathing through the nose
• Easy bruising
• Frequent or repeated nosebleeds
• Hearing loss
• Nasal discharge, usually bloody
• Prolonged bleeding
• Stuffy nose

Exams and Tests

The doctor may see the angiofibroma when examining the upper throat.

Tests that may be done include:

• CT scan of the head
• Nasal mucosal biopsy (this can be very dangerous, and should only be done after using CT scan or arteriogram to see the blood supply to the growth)
• X-ray

Treatment

Treatment is required if the angiofibroma is growing larger, blocking the airways, or causing repeated nosebleeds. In some cases, no treatment is necessary.

Surgery may be needed to remove the tumor. Removal is often difficult because the tumor is not enclosed and may have spread deeply to other areas.

A procedure called embolization may be done to prevent the tumor from bleeding. The procedure may correct the nosebleeds by itself, or it may be followed by surgery to remove the tumor.

Outlook (Prognosis)

Although not cancerous, angiofibromas may continue to grow. Some may disappear on their own.

It is common for the tumor to return after surgery.

Possible Complications

• Anemia
• Pressure on the brain (rare)
• Spread of the tumor to the nose, sinuses, and other structures

When to Contact a Medical Professional

Call your health care provider if you often have nosebleeds.

Prevention

There is no known way to prevent this condition.

References

Cummings CW, Flint PW, Haughey BH, et al. Otolaryngology: Head & Neck Surgery. 4th ed. St Louis, Mo: Mosby; 2005.

Grainger RC, Allison D, Adam, Dixon AK. Diagnostic Radiology: A Textbook of Medical Imaging. 4th ed. Orlando, Fl: Churchill Livingstone; 2001.

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