Dermatology Online Journal

Yellow nails syndrome in two siblings
Thaer Douri
Dermatology Online Journal 14 (9): 7

Ministry of Health, Syria-Hama. s-dori@scs-net.org

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Abstract

Yellow nails syndrome (YNS) is a rather rare condition. The syndrome was first described in 1964 by Samman and White. Later, Emerson added pleural effusion as a frequent feature of the disease. The classic triad of YNS includes yellow nails, lymphedema, and respiratory tract involvement, with or without sinusitis. However, these three alterations are simultaneously present in only 27 percent of cases. We discribe two siblings (ages 32 and 26) with 20 yellow nails. There have been only a few published reports where a positive family history (FH) has been documented in cases of YNS.

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Case reports

Figure 1	Figure 2

Figure 3

Case 1: A 32-year-old female had 20 yellow nails from childhood (Fig. 1). There are indurated and nonpitting edema in the lower legs (Fig. 2) and verrucous hyperplasia (Fig. 3). No respiratory tract involvement or sinusitis were present.

Figure 4	Figure 5

Case 2: A 26-year-old female had 20 yellow nails from childhood (Figs. 4 & 5) without lymphedema, respiratory tract involvement, or sinusitis.

Figure 6

Other family members were healthy. In both cases all 20 nails showed a yellowish-green discoloration with thickening of the nail plates and lack of cuticles (Fig. 6). The linear nail growth was markedly reduced and onycholysis was present. In Case 1 there is lymphedema; there is no respiratory tract involvement in either case. Pulmonary features and lymphedema may not occur until late in the course, therefore follow-up of patients who present with this type of nail dystrophy is recommended. We treated both patients with VE 1000 IU L/day and noted some improvement manifesting as some decrease in the thickening of the nails and some improvement in color.

Discussion

Yellow nails syndrome (YNS) is a rather rare condition. This syndrome was first described in 1964 by Samman and White [1]. Later, Emerson [2] added pleural effusion as a frequent feature of the disease. The classic triad of YNS includes yellow nails, lymphedema, and respiratory tract involvement, with or without sinusitis. However, these three abnormalities are simultaneously present in only 27 percent of cases [3]. A positive family history has been documented in just a few reports [4].

The characteristics of involved nails include thickening, transverse ridging, diminished growth, increased curvature with a "hump," and onycholysis. The curvature is exaggerated in both the transverse and longitudinal directions. The lunulae and cuticles may be absent. The color may vary from pale yellow to green. The etiology is not certain, but nail changes have been postulated to be secondary to congenitally hypoplastic lymphatics [5]. The nail growth is slow (0.1-0.25mm/week, normal 0.5-2mm/week) [6].

In a series of 97 patients reviewed by Nordkild in 1986 [7], yellow nails were present in 99 percent of the cases and were the first symptom in 37 percent. Lymphedema was present in 80 percent and was the first symptom in 34 percent. Respiratory tract involvement (mainly recurrent attacks of chronic bronchitis occasionally associated with bronchiectases, chronic sinusitis, pneumonia or pleuritis) was present in 63 percent; it was the first symptom in 29 percent. Yellow nails syndrome is most often seen in middle-aged individuals; there is a male/female ratio of 1/1.6 [7].

Patients often give a history of recurrent attacks of bronchitis and may have chronic sinusitis, bronchiectasis, and recurrent pneumonia [13]. Of the twelve patients reported from the Mayo Clinic, eight had recurrent pleural effusion and five had bronchiectasis; in this series, the first manifestation of the syndrome was either lymphedema or yellow nails. Pleural effusion appeared somewhat later in all cases. The pleural effusion is usually exudative (idiopathic, infectious, or chylothorax). The pleural fluid characteristically contains a high percentage of lymphocytes [12].

Yellow nails syndrome has been reported in association with thyroid disease [7], hypogammaglobulinemia [7], nephrotic syndrome [7], protein-losing enteropathy [8], obstructive sleep apnea [9], and xanthogranulomatous pyelonephritis [10].

There have been only a few published reports of YNS in which a positive family history has been documented [4]. Razi described a patient with familial YNS associated with pulmonary findings, lymphedema, and hypoproteinemia in a 70-year-old woman; manifestations of this syndrome were also present in her three siblings [13]. Finegold et al. found mutations in the FOXC2 gene (forkhead transcription factor gene) in 11 families with various lymphedema syndromes, one of which was YNS [11]. In our cases other family members did not have any of the typical abnormalities.

The treatment for YNS is mostly symptomatic. However, intralesional corticosteroid injections, oral vitamin E 1200 IU/day, topical vitamin E, and pulse itraconazole 400mg/day for one week per month have been reported to be useful [6]. We treated with vitamin E 1000 IU L/day and noted some improvement.

References

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10. Danenberg HD, Eliashar R, Flusser G, Rosenmann E, Chajek-Shault. Yellow nail syndrome and xanthogranulomatous pyelonephritis. Postgrad Med J. 1995 Feb;71[832]:110-1 PubMed [PubMed - indexed for MEDLINE]

11. Finegold DN, Kimak MA, Lawrence EC, Levinson KL, Cherniske EM, Pober BR, Dunlap JW, Ferrell RE. Truncating mutations in FOXC2 cause multiple lymphedema syndromes. Hum Mol Genet. 2001 May 15;10[11]:1185-9. PubMed [PubMed - indexed for MEDLINE]

12. Solal - Celigny P, Cormier Y, Fournier M. The yellow nail syndrome. Light and electron microscopic aspects of the pleura. Arch pathol Lab Med 1983; 107: 183-85. PubMed [PubMed - indexed for MEDLINE]

13. Razi E. Familial yellow nail syndrome. Dermatol Online J. 2006 Feb 28;12[2]:15. PubMed [PubMed - indexed for MEDLINE]

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