About pierre-marie's disease
What is pierre-marie's disease?
Cicatricial alopecias are classified as primary or secondary. This discussion is confined to the primary cicatricial alopecias, in which the hair follicle is the target of a destructive inflammatory process. In secondary cicatricial alopecias, destruction of the hair follicle is incidental to a non-follicle-directed process or external injury, such as severe infections, burns, radiation, or tumors.
Primary cicatricial alopecia refers to a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. Hair loss can be gradual, without symptoms, and unnoticed for long periods. In other cases, the hair loss may be associated with severe itching, pain and burning, and progress rapidly. Cicatricial alopecia occurs in otherwise healthy men and women of all ages and is seen worldwide.
What are the symptoms for pierre-marie's disease?
The symptoms of acromegaly can be difficult to detect since they usually develop slowly over time. For example, you may notice over a period of several months that you have a ring that feels increasingly tight on your finger and one day it no longer fits. You may also find that you need to go up a size in shoes if you have this condition.
Common symptoms of acromegaly are:
- enlarged bones in the face, feet, and hands
- excessive hair growth in women
- an enlarged jaw or tongue
- a prominent brow
- excessive growth spurts, which are more common in people who’ve had abnormal growth before adolescence
- weight gain
- swollen and painful joints that limit movement
- spaces between the teeth
- splayed fingers and toes
- a hoarse, deep voice
- an inability to sleep
- muscle weakness
- profuse sweating
- body odor
- enlarged sebaceous glands, which are glands that produce oils in the skin
- thickened skin
- skin tags, which are noncancerous growths
You should see your doctor if you’ve noticed one or more of these symptoms.
What are the causes for pierre-marie's disease?
GH is part of a group of hormones that regulate the growth and development of the body. People with acromegaly have too much GH. It accelerates bone growth and organ enlargement. Because of this growth stimulation, the bones and organs of people with acromegaly are much larger than other people’s bones and organs.
GH is made in the brain’s pituitary gland. According to the National Institutes of Health (NIH), more than 95 percent of people with acromegaly have a benign tumor affecting their pituitary. This tumor is called an adenoma. Adenomas are common. They affect about 17 percent of people. In most people, these tumors don’t cause excess GH, but when they do can result in acromegaly.
What are the treatments for pierre-marie's disease?
Treatment for acromegaly is based on your age and overall health. The goals of treatment are to:
- bring GH production levels back to normal
- relieve pressure around any growing pituitary tumors
- maintain normal pituitary function
- treat any hormone deficiencies and improve the symptoms of acromegaly
Several types of treatments may be needed.
Surgery to remove the tumor causing excess GH is the first option doctors usually recommend to people with acromegaly. Usually, this treatment is quick and effective in reducing GH levels, which can improve symptoms. One possible complication is damage to the pituitary tissues that surrounded the tumor. If this happens, it can mean you’ll need to start a lifelong pituitary hormone replacement treatment. Rare but serious complications include cerebrospinal fluid leaks and meningitis.
Medication is another treatment option that’s often used if surgery isn’t successful in reducing GH levels, and it can also be used to shrink large tumors before surgery. These types of medications are used to regulate or block GH production:
- somatostatin analogs
- GH receptor antagonists
- dopamine agonists
Radiation may be used to destroy large tumors or sections of tumor left after surgery or when medications alone aren’t effective. Radiation can slowly help to lower GH levels when used along with medication. A dramatic decrease in GH levels using this type of treatment may take several years, with radiation administered in multiple four- to six-week sessions. Radiation can impair your fertility. In rare cases, it can lead to vision loss, brain injury, or secondary tumors.
What are the risk factors for pierre-marie's disease?
Acromegaly can start any time after puberty. However, it occurs more often in middle age. People aren’t always aware of their condition. Changes to the body may take place slowly over the course of many years.
Is there a cure/medications for pierre-marie's disease?
The outlook for people with acromegaly is usually positive if the condition is discovered in the early stages. Surgery to remove pituitary tumors is usually successful. Treatment may also help keep acromegaly from having long-term effects.
Coping with the symptoms and treatments of acromegaly can be challenging. Many people find it helpful to join support groups. Check online to find local support groups near you.