Yellow nail syndrome
The yellow nail syndrome (YNS) is a rare disorder principally affecting the lymphatic system.
It is characterized by a clinical triad:
- nail discolouration: yellow slow-growing dystrophic nails (scleronychia)
- lymphedema (peripheral/primary)
- pulmonary disease: see yellow nail syndrome (pulmonary manifestations)
Other associated features include:
It is a rare disease with an approximate incidence of less than one million/year 8. There may be a slightly increased female predilection 5. While clinical onset varies from birth to late adult life, it is thought to typically affect those in early middle age 5.
The syndrome can sometimes be associated with
Clinical features tend to be variable in severity amongst affected individuals and temporal variation is common.
The exact pathogenesis is not well understood although anatomical or functional lymphatic drainage abnormalities have been proposed as an underlying mechanism.
Imaging is often performed in the evaluation of pulmonary manifestations.
On CT chest, while individual features are non-specific, there may be evidence of
- sequelae of recurrent pulmonary infection
- pleural effusions
Treatment and prognosis
While there is no specific treatment for the syndrome, however some patients are helped with biotin and vitamin E supplementation 8. In addition most patients are often managed with supportive measures targeted at ameliorating the various clinical manifestations (e.g. treating respiratory infections).
The general clinical course is benign but is somewhat dependent on disease associations. In 10-30% cases there is spontaneous resolution of the condition 8.
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- 3. Fields CL, Roy TM, Ossorio MA et-al. Yellow nail syndrome: a perspective. J Ky Med Assoc. 1992;89 (11): 563-5. Pubmed citation
- 4. Hoque SR, Mansour S, Mortimer PS. Yellow nail syndrome: not a genetic disorder? Eleven new cases and a review of the literature. Br. J. Dermatol. 2007;156 (6): 1230-4. doi:10.1111/j.1365-2133.2007.07894.x - Pubmed citation
- 5. Nordkild P, Kromann-Andersen H, Struve-Christensen E. Yellow nail syndrome-the triad of yellow nails, lymphedema and pleural effusions. A review of the literature and a case report. Acta Med Scand. 1986;219 (2): 221-7. Pubmed citation
- 6. Venencie PY, Dicken CH. Yellow nail syndrome: report of five cases. J. Am. Acad. Dermatol. 1984;10 (2 Pt 1): 187-92. Pubmed citation
- 7. Penneys N. Skin Manifestations Of Aids, Second Edition. CRC Press. (1995) ISBN:1853172421. Read it at Google Books - Find it at Amazon
- 8. Lübbert C, Kunz M. Yellow Nail Syndrome. (2017) Deutsches Arzteblatt international. 114 (50): 874. doi:10.3238/arztebl.2017.0874b - Pubmed