Milroy disease

Milroy's disease
Classification and external resources
ICD-10 9 OMIM DiseasesDB eMedicine MeSH GeneReviews

Milroy's disease is a familial disease characterised 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

The defect in Milroy's disease is present from birth and symptoms are usually first experienced in childhood. The most common problem is one-sided leg swelling, unilateral edema, which is progressive and can affect both legs. Impaired intestinal lymphatics can cause steatorrhea due to impaired transport of chylomicrons. Defective lymphocyte circulation can cause lymphopenia and impaired cell-mediated immunity.

This disease is more common in women and an association with the gene FLT4 has been described.[6]

See also

References

External links

  • GeneReview/NIH/UW entry on Milroy Disease