Milroy's disease

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Milroy's disease
Classification and external resources
ICD-10 Q82.0
ICD-9 757.0
OMIM 153100
DiseasesDB 8228
eMedicine med/1482
MeSH D008209
GeneReviews

Milroy's disease is a familial disease characterized by lymphedema, commonly in the legs, caused by congenital abnormalities in the lymphatic system. Disruption of the normal drainage of lymph leads to fluid accumulation and hypertrophy of soft tissues.[1][2] It is also known as Milroy disease, Nonne-Milroy-Meige syndrome and hereditary lymphedema.[3]

It was named by Sir William Osler for William Milroy, an American physician, who described a case in 1892, though it was first described by Rudolf Virchow in 1863.[4][5]

Description[edit]

The most common presentation of Milroy Disease is bilateral lower extremity lymphedema, and may also be accompanied by hydrocele.[6]

This disease is more common in women and an association with the gene FLT4 has been described.[7] FLT4 codes for VEGFR-3, which is implicated in development of the lymphatic system.

See also[edit]

References[edit]

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. p. 849. ISBN 0-7216-2921-0. 
  2. ^ David Lowell Strayer; Raphael Rubin (2007). Rubin's Pathology: Clinicopathologic Foundations of Medicine 5th Edition. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-9516-8. 
  3. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  4. ^ synd/1326 at Who Named It?
  5. ^ W. F. Milroy. An undescribed variety of herditary edema. New York Medical Journal, 1892, 56: 505-508.
  6. ^ "Milroy Disease". United States Library of Medicine. Retrieved March 2014. 
  7. ^ Spiegel R, Ghalamkarpour A, Daniel-Spiegel E, Vikkula M, Shalev SA (2006). "Wide clinical spectrum in a family with hereditary lymphedema type I due to a novel missense mutation in VEGFR3". Journal of Human Genetics 51 (10): 846–50. doi:10.1007/s10038-006-0031-3. PMID 16924388. 

External links[edit]