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LYMPHEDEMA PRAECOX MEIGE SYNDROME

Lymphedema Praecox

Lymphedema Meige Syndrome

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MEIGE LYMPHEDEMA SYNDROME

Lymphedema Praecox

See also: Nonne-Milroy-Meige Syndrome, Meige's lymphedema, Hereditary lymphedema II, familial lymphedema praecox

See also http://www.lymphedemapeople.com/thesite/all_about_lymphedema.htm

Form of primary hereditary lymphedema that starts at or around the time of puberty. The affected limbs are generally the legs.  

Named after French physician Dr. Henri Meige who first described hereditary lymphedema in 1891. This form of lymphedema which usually presents itself at or during puberty is the most common of the hereditary lymphedemas, account for 65-80% of all diagnosed cases.

Meige-Type Lymphedema

Also known as Lymphedema II, this syndrome is similar to Lymphedema I but the onset of peripheral edema occurs during the second to the fifth decades. The legs are the most commonly involved, and lymphangiography reveals hypoplasia of peripheral lymphatics with dilation of lymphatic trunks.

Diagnosis

Basic diagnosis can be made by the fact that swelling (generally of the legs) presents during puberty  and there is a family history of similar swelling.  Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.

Etiology

The cause of Lymphedema Praecox is a break in the VEGFR3 gene. The gene FOXC2 is implicated and there is also suspect third lymphedema gene. 

Complications

The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections).  Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.

Other Indications

Related conditions may also include yellow nail syndrome, pulmonary hypertension, cerebrovascular malformations and distichiasis

Treatment

Decongestive therapy is the most widely accepted form of treatment.  There is no cure for Lymphedema praecox but the condition can be managed by early diagnosis and treatment.

Prognosis

Long term prognosis is excellent if  the condition is identified early and treatment begins so after the diagnosis is made.

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Meige Lymphedema 

Stages

LATENCY STAGE
Lymphatic transport capacity is reduced
No visible/palpable edema
Subjective complaints are possible

STAGE I
(Reversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting edema
Reduces with elevation (no fibrosis)

STAGE II
(Spontaneously Irreversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting becomes progressively more difficult
Connective tissue proliferation (fibrosis)

STAGE III
(Lymphostatic Elephantiasis)
Accumulation of protein rich edema fluid
Non pitting
Fibrosis and sclerosis (severe induration)
Skin changes (papillomas, hyperkeratosis, etc.)

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Familial lymphedema praecox: Meige's disease

abstract

http://www.ncbi.nlm.nih.gov/pubmed?term=Familial%20lymphedema%20praecox%3A%20Meige's%20disease

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MEIGE LYMPHEDEMA, LYMPHEDEMA, LATE-ONSET, LYMPHEDEMA PRAECOX

#153200 LYMPHEDEMA, HEREDITARY, II

Online Mendelian Inheritance in Man

John Hopkins University

MEIGE LYMPHEDEMA
LYMPHEDEMA, LATE-ONSET
LYMPHEDEMA PRAECOX

Gene map locus 16q24.3

A number sign (#) is used with this entry because of evidence that hereditary lymphedema type II is caused by mutation in the forkhead family transcription factor gene MFH1 (FOXC2; 602402). Allelic disorders with overlapping features include the lymphedema-distichiasis syndrome (153400), lymphedema and ptosis (153000), and lymphedema and yellow nail syndrome (153300). Also see hereditary lymphedema type I, or Milroy disease (153100).

CLINICAL FEATURES

Edema, particularly severe below the waist, develops about the time of puberty. Meige (1898) described 8 cases in 4 generations without male-to-male transmission. Goodman (1962) reported the condition in 2 sisters and a brother with presumed normal parents who were not known to be related. Herbert and Bowen (1983) described a kindred with many cases of lymphedema of postpubertal onset. Involvement of the upper limbs (as well as the lower limbs), face, and larynx and, in one, a persistent pleural effusion were notable features. Scintilymphangiography indicated paucity or absence of lymph nodes in the axillae and above the inguinal ligaments. Chronic facial swelling resulted in a characteristic appearance of affected members including puffiness, shiny skin, deep creases, and, in some, excessive wrinkling. Emerson (1966) noted similar facial features and remarked on the possible erroneous diagnosis of myxedema.

Herbert and Bowen (1983) noted the difficulties of nosology. For example, because lymphedema and yellow nail syndrome has yellow or dystrophic nails as a variable feature, this could be the same disorder. They pointed also to the association of late-onset lymphedema with deafness (Emberger et al., 1979) and with primary pulmonary hypertension and cerebrovascular malformations (152900; Avasthey and Roy, 1968).

Figueroa et al. (1983) reported the association of cleft palate. In their family, the mother, with only lymphedema praecox of the legs, gave birth to 5 sons, 3 of whom had both lymphedema of the legs and cleft palate. A mild form of lymphedema affecting mainly the medial aspect of both ankles in a 21-year-old son was pictured.

Andersson et al. (1995) described a family in which 3 individuals, a grandmother, her son and her grandson, had onset of lymphedema in their mid-20s or 30s. The grandson was 23 years old when he had his first episode of lymphedema, which was thought to be due to thrombophlebitis. During the ensuing decade, he had episodic waxing and waning of lymphedema of both lower limbs and was treated with anticoagulant therapy. At the age of 35, he developed lymphangiosarcoma on the inner right thigh and died of metastases some months later. Lymphangiosarcoma, usually associated with postmastectomy lymphedema, had not been described previously in late-onset hereditary lymphedema. Andersson et al. (1995) raised the question of whether a genetic predisposition to malignancy combined with the lymphedema was etiologically significant. There seemed to be an unusually high frequency of cancer (uterine, colon, lung, prostate, breast, and bone) in the proband's family

MOLECULAR GENETICS

Finegold et al. (2001) found a mutation in the FOXC2 gene (602402.0007) in a family with Meige lymphedema and also in a family with yellow nail syndrome.

HETEROGENEITY

Finegold et al. (2001) noted that the phenotypic classification of dominantly inherited lymphedema includes Milroy disease (hereditary lymphedema I), Meige lymphedema (hereditary lymphedema II), lymphedema-distichiasis syndrome, lymphedema and ptosis, and yellow nail syndrome. The phenotypes reported in their 11 families overlapped the findings reported in Meige lymphedema, lymphedema-distichiasis syndrome, lymphedema and ptosis, and yellow nail syndrome, but not in Milroy disease. Milroy disease is associated with mutation in the FLT4 gene (136352), whereas mutations in the FOXC2 gene were observed in the 4 lymphedema syndromes that had phenotypic overlap

REFERENCES

1. Andersson, H. C.; Parry, D. M.; Mulvihill, J. J. :
Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications. Am. J. Med. Genet. 56: 72-75, 1995.
PubMed ID : 7747790
2. Avasthey, P.; Roy, S. B. :
Primary pulmonary hypertension, cerebrovascular malformation, and lymphoedema of the feet in a family. Brit. Heart J. 30: 769-775, 1968.
PubMed ID : 5718986
3. Emberger, J. M.; Navarro, M.; Dejean, M.; Izarn, P. :
Surdi-mutite, lymphoedeme des membres inferieurs et anomalies hematologiques (leucose aigue cytopenies) a transmission autosomique dominante. J. Genet. Hum. 27: 237-245, 1979.
PubMed ID : 295075
4. Emerson, P. A. :
Yellow nails, lymphoedema, and pleural effusions. Thorax 21: 247-253, 1966.
PubMed ID : 5914998
5. Figueroa, A. A.; Pruzansky, S.; Rollnick, B. R. :
Meige disease (familial lymphedema praecox) and cleft palate: report of a family and review of the literature. Cleft Palate J. 20: 151-157, 1983.
PubMed ID : 6342849
6. Finegold, D. N.; Kimak, M. A.; Lawrence, E. C.; Levinson, K. L.; Cherniske, E. M.; Pober, B. R.; Dunlap, J. W.; Ferrell, R. E. :
Truncating mutations in FOXC2 cause multiple lymphedema syndromes. Hum. Molec. Genet. 10: 1185-1189, 2001.
PubMed ID : 11371511
7. Goodman, R. M. :
Familial lymphedema of the Meige's type. Am. J. Med. 32: 651-656, 1962
8. Herbert, F. A.; Bowen, P. A. :
Hereditary late-onset lymphedema with pleural effusion and laryngeal edema. Arch. Intern. Med. 143: 913-915, 1983.
PubMed ID : 6679236
 
9. Juchems, R. :
Das hereditaere Lymphoedem, Typ Meige. Klin. Wschr. 41: 328-332, 1963.
10. Meige, H. :
Dystrophie oedemateuse hereditaire. Presse Med. 6: 341-343, 1898.
11. Osterland, G. :
Beobachtungen zum Nonne-Milroy-Meige-Syndrom. Z. Menschl. Vererb. Konstitutionsl. 36: 108-117, 1961.
PubMed ID : 14482593
12. Wheeler, E. S.; Chan, V.; Wassman, R.; Rimoin, D. L.; Lesavoy, M. A. :
Familial lymphedema praecox: Meige's disease. Plast. Reconst. Surg. 67: 362-364, 1981.
PubMed ID : 7232571

CONTRIBUTORS

George E. Tiller - updated : 10/22/2001

CREATION DATE

Victor A. McKusick : 6/2/1986

EDIT HISTORY

carol : 11/19/2003

http://omim.org/entry/153200

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External Links:

Meige Lymphedema Genetics Home Reference 

http://ghr.nlm.nih.gov/condition/meige-lymphedema

Lymphedema Praecox

http://dermatology.cdlib.org/1508/articles/2008111801/a07.html 

Meige disease (familial lymphedema praecox) and cleft palate: report of a familyand review of the literature.

http://www.ncbi.nlm.nih.gov/pubmed?term=Familial%20lymphedema%20praecox%20meige%3A%20

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Updated Dec. 26, 2011