(Chest. 2000;117:1516-1518.)
© 2000
American College of Chest Physicians
Yellow Nail Syndrome*
Resolution of Yellow Nails After Successful Treatment of Breast Cancer
Mobeen Iqbal, MD;
Leonard J. Rossoff, MD;
Kamel A. Marzouk, MD and
Harry N. Steinberg, MD
*
From the Division of Pulmonary and Critical Care Medicine, Long Island Jewish Medical Center, New Hyde Park, NY.
Correspondence to: Leonard J. Rossoff, MD, Division of Pulmonary and Critical Care Medicine, Long Island Jewish Medical Center, The Long Island Campus of the Albert Einstein College of Medicine, Room C-20, 270–05 76th Ave, New Hyde Park, NY 11042
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Abstract
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Yellow nail syndrome (YNS) is a rare entity of unknown cause
in which congenitally hypoplastic lymphatics play a major role in the
clinical manifestations of the disease. YNS has been associated with
many malignancies and immune disorders. We report a case of new-onset
YNS associated with breast cancer and dramatic improvement in the
yellow nails with cancer treatment.Key words:
carcinoma of breast; chemotherapy; neoplasm; yellow nail
Key Words: carcinoma of breast • chemotherapy • neoplasm • yellow nail
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Introduction
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Yellow
nail syndrome (YNS) is a rare disorder, characterized by
rhinosinusitis, pleural effusions, bronchiectasis, lymphedema, and
dystrophic yellow nails.1
2
The classic triad, described
by Emerson, of lymphedema, slow-growing yellow nails, and
pleural effusion is seen in only one third of patients.3
4
More than 150 cases attributed to congenitally hypoplastic lymphatics
are reported in the literature. Individual manifestations of the
syndrome can appear at different times, and clinical onset varies from
birth to late adult life.5
YNS has been associated with
autoimmune disorders, such as thyroiditis, systemic lupus
erythematosus, and rheumatoid arthritis.5
There are also
isolated case reports of YNS associated with malignancies in cancer of
the breast,5
larynx,6
lung,7
endometrium,8
gall bladder,9
metastatic
sarcoma,10
metastatic melanoma,11
Hodgkin’s
disease,12
and mycosis fungoides.13
It has
also been described in tuberculosis, AIDS, and other immunodeficiency
states, and with the use of certain drugs.14
We report what we believe to be only the second case of breast cancer
in which the yellow nails remitted after treatment.5
In
this case, unlike the previous one, nail changes were restricted to the
upper extremities.
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Case Report
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A 62-year-old woman presented with chronic cough of 1.5 years’
duration. The cough was worse in the morning and intermittently
productive of purulent sputum. Her pulmonary symptoms transiently
responded to antibiotics but recurred about 2 to 3 weeks after their
cessation. She also complained of chronic nasal congestion and
postnasal drip but denied wheezing, skin disease, or other evidence of
atopy and reported only one remote episode of lobar pneumonia. She also
noted progressive yellowing of the nailbeds of both hands for several
months before presentation. She denied corticosteroid use or evidence
of immune deficits. There was no history of swallowing dysfunction, and
her weight and appetite remained stable. She was on thyroid replacement
therapy subsequent to a thyroidectomy (1964) for goiter and
Hashimoto’s thyroiditis. She ceased smoking 2 years before, with a
total of 10 to 15 pack-years. Her family history was unremarkable.
On physical examination, she was found to have dystrophic yellow nails
in both hands (Fig 1
) with normal toenails. Chest auscultation revealed bilateral,
scattered, coarse crackles in the lower zones. There was no evidence of
peripheral edema. The rest of the examination was not revealing. CT
scan of the sinuses showed opacification of both maxillary sinuses and
mucosal thickening of both sphenoid sinuses. High-resolution CT scan of
the chest (Fig 2
) showed mild cylindrical bronchiectasis mainly in the right lower lobe
with subsegmental atelectasis in the right middle lobe and lingula.
Also seen on this image was an unsuspected mass in the right breast
measuring 1 x 1.5 cm with associated axillary lymphadenopathy.
Excisional biopsy revealed an infiltrating ductal carcinoma of the
breast positive for estrogen and progesterone receptors. Axillary lymph
node dissection confirmed tumor nodal involvement (T1N1M0). She was
treated with monthly cycles of cyclophosphamide, methotrexate, and
5-flurouracil for 8 months. She quickly noted improvement in her
dystrophic yellow nails, which appeared normal by the end of the
chemotherapeutic regimen (Fig 1)
. She subsequently received radiation
therapy and tamoxifen. In subsequent 2 year follow-up, there is no
recurrence of tumor or yellow nails.
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Discussion
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Since the original description by Samman and White,15
many associations of YNS have been described. Airway manifestations
include rhinosinusitis and bronchiectasis. Yellow nails result from
slow growth, possibly secondary to defective lymphatic drainage. The
nails become dystrophic with longitudinal or transverse ridging and
loss of lunula and cuticles.16
Pleural effusions appear to
be a later manifestation of the syndrome secondary to inadequate
drainage by overstressed hypoplastic lymphatics rather than increased
fluid production.17
The cause of bronchiectasis is
unclear, but again, dysfunctional lymphatics are thought to play an
important role with compromised drainage of secretions and local immune
function.18
Various malignancies have been associated with YNS, and one case of the
yellow nails improved dramatically after resection of a laryngeal
cancer.19
As in our case, Gupta et al5
reported similar improvement after surgery and chemotherapy for a
carcinoma of the breast. Interestingly, improvement was seen in the
fingernails only. Although partial or complete improvement in the nails
may occur spontaneously in up to one third of patients, the temporal
relationship and pace of the improvement strongly favors an association
with successful treatment of malignancy. Possible explanations include
direct involvement by tumor of already stressed and dysfunctional
lymphatics or the elaboration of mediators such as peptide hormones
that inhibit lymphatic function.7
Thus, yellow nails may
be a paraneoplastic manifestation of cancer that may resolve with
effective treatment. The diagnosis of YNS should raise the index of
suspicion for malignancy and other associated diseases.
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Footnotes
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Abbreviation: YNS = yellow nail syndrome
Received for publication July 20, 1999.
Accepted for publication October 18, 1999.
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References
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Guin, JD, Elleman, JH (1979) Yellow nail syndrome: possible association with malignancy. Arch Dermatol 115,734-735
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Abstract
Introduction
