Primary lymphedema: Difference between revisions
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{{Infobox medical condition | |||
| name = Primary lymphedema | |||
| synonyms = [[Congenital lymphedema]], [[hereditary lymphedema]] | |||
| field = [[Vascular medicine]], [[Genetics]] | |||
| symptoms = [[Swelling]] in the [[limbs]], [[skin thickening]], [[pain]] | |||
| complications = [[Infection]], [[lymphangiosarcoma]] | |||
| onset = [[Birth]] or [[puberty]] | |||
| duration = [[Chronic]] | |||
| causes = [[Genetic mutation]] | |||
| risks = [[Family history]] | |||
| diagnosis = [[Physical examination]], [[imaging studies]] | |||
| differential = [[Secondary lymphedema]], [[lipedema]] | |||
| treatment = [[Compression therapy]], [[manual lymphatic drainage]], [[surgery]] | |||
| prognosis = [[Variable]] | |||
| frequency = Rare | |||
}} | |||
'''Primary lymphedema''' is a type of [[lymphedema]] that is present at birth or develops later in life without any apparent cause. It is a rare, inherited condition characterized by swelling (lymphedema) of certain parts of the body. | '''Primary lymphedema''' is a type of [[lymphedema]] that is present at birth or develops later in life without any apparent cause. It is a rare, inherited condition characterized by swelling (lymphedema) of certain parts of the body. | ||
== Causes == | == Causes == | ||
Primary lymphedema is caused by the malformation or malfunction of the [[lymphatic system]], which is part of the body's immune system. The lymphatic system produces and transports lymph fluid from tissues to the bloodstream. In primary lymphedema, lymph fluid does not drain properly and accumulates causing swelling in the affected areas. | Primary lymphedema is caused by the malformation or malfunction of the [[lymphatic system]], which is part of the body's immune system. The lymphatic system produces and transports lymph fluid from tissues to the bloodstream. In primary lymphedema, lymph fluid does not drain properly and accumulates causing swelling in the affected areas. | ||
== Types == | == Types == | ||
There are three types of primary lymphedema: | There are three types of primary lymphedema: | ||
* '''Congenital lymphedema''' - This type is evident at birth or within the first two years of life. | * '''Congenital lymphedema''' - This type is evident at birth or within the first two years of life. | ||
* '''Lymphedema praecox''' - This type occurs after the age of two but before the age of 35, and it is the most common form of primary lymphedema. | * '''Lymphedema praecox''' - This type occurs after the age of two but before the age of 35, and it is the most common form of primary lymphedema. | ||
* '''Lymphedema tarda''' - This type occurs after the age of 35. | * '''Lymphedema tarda''' - This type occurs after the age of 35. | ||
== Symptoms == | == Symptoms == | ||
The main symptom of primary lymphedema is persistent swelling, usually in the arms or legs, but it can also affect other parts of the body. Other symptoms may include a feeling of heaviness, discomfort, repeated skin infections, and thickening and hardening of the skin in the affected areas. | The main symptom of primary lymphedema is persistent swelling, usually in the arms or legs, but it can also affect other parts of the body. Other symptoms may include a feeling of heaviness, discomfort, repeated skin infections, and thickening and hardening of the skin in the affected areas. | ||
== Diagnosis == | == Diagnosis == | ||
Primary lymphedema is diagnosed based on the patient's symptoms and medical history. Imaging tests such as [[MRI]], [[CT scan]], and [[lymphoscintigraphy]] may be used to visualize the lymphatic system and confirm the diagnosis. | Primary lymphedema is diagnosed based on the patient's symptoms and medical history. Imaging tests such as [[MRI]], [[CT scan]], and [[lymphoscintigraphy]] may be used to visualize the lymphatic system and confirm the diagnosis. | ||
== Treatment == | == Treatment == | ||
There is no cure for primary lymphedema. Treatment focuses on managing the symptoms and includes [[compression therapy]], [[manual lymphatic drainage]], and [[exercise therapy]]. In some cases, surgery may be considered. | There is no cure for primary lymphedema. Treatment focuses on managing the symptoms and includes [[compression therapy]], [[manual lymphatic drainage]], and [[exercise therapy]]. In some cases, surgery may be considered. | ||
== See also == | == See also == | ||
* [[Secondary lymphedema]] | * [[Secondary lymphedema]] | ||
| Line 26: | Line 36: | ||
* [[Lymphedema praecox]] | * [[Lymphedema praecox]] | ||
* [[Lymphedema tarda]] | * [[Lymphedema tarda]] | ||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Lymphatic system disorders]] | [[Category:Lymphatic system disorders]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
{{stub}} | {{stub}} | ||
{{dictionary-stub1}} | {{dictionary-stub1}} | ||
{{No image}} | {{No image}} | ||
Latest revision as of 03:57, 4 April 2025
| Primary lymphedema | |
|---|---|
| Synonyms | Congenital lymphedema, hereditary lymphedema |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Swelling in the limbs, skin thickening, pain |
| Complications | Infection, lymphangiosarcoma |
| Onset | Birth or puberty |
| Duration | Chronic |
| Types | N/A |
| Causes | Genetic mutation |
| Risks | Family history |
| Diagnosis | Physical examination, imaging studies |
| Differential diagnosis | Secondary lymphedema, lipedema |
| Prevention | N/A |
| Treatment | Compression therapy, manual lymphatic drainage, surgery |
| Medication | N/A |
| Prognosis | Variable |
| Frequency | Rare |
| Deaths | N/A |
Primary lymphedema is a type of lymphedema that is present at birth or develops later in life without any apparent cause. It is a rare, inherited condition characterized by swelling (lymphedema) of certain parts of the body.
Causes[edit]
Primary lymphedema is caused by the malformation or malfunction of the lymphatic system, which is part of the body's immune system. The lymphatic system produces and transports lymph fluid from tissues to the bloodstream. In primary lymphedema, lymph fluid does not drain properly and accumulates causing swelling in the affected areas.
Types[edit]
There are three types of primary lymphedema:
- Congenital lymphedema - This type is evident at birth or within the first two years of life.
- Lymphedema praecox - This type occurs after the age of two but before the age of 35, and it is the most common form of primary lymphedema.
- Lymphedema tarda - This type occurs after the age of 35.
Symptoms[edit]
The main symptom of primary lymphedema is persistent swelling, usually in the arms or legs, but it can also affect other parts of the body. Other symptoms may include a feeling of heaviness, discomfort, repeated skin infections, and thickening and hardening of the skin in the affected areas.
Diagnosis[edit]
Primary lymphedema is diagnosed based on the patient's symptoms and medical history. Imaging tests such as MRI, CT scan, and lymphoscintigraphy may be used to visualize the lymphatic system and confirm the diagnosis.
Treatment[edit]
There is no cure for primary lymphedema. Treatment focuses on managing the symptoms and includes compression therapy, manual lymphatic drainage, and exercise therapy. In some cases, surgery may be considered.
