Yellow Nail Syndrome

the tip of one central venous catheter, but this was not associated with bacteremia. The colony count for pneumococcus was also not specified. Groeger and colleagues12 described the port pocket infection of an implanted infusion port device by S pneumoniae, but there was no associated bacteremia. Thus, it seems that pneumococcus can colonize and infect various forms of IV catheters and devices. However, after an extensive literature search through MEDLINE, to the best of our knowledge, an IV catheter has never been implicated as a source of pneumococcal bacteremia. Our patient received total parenteral nutrition through this central venous catheter for 6 days. Many of the central venous catheters mentioned in previous studies were used for parenteral nutrition. Even in studies of catheters used for total parenteral nutrition only, there were no cases of catheter-tip infection or bacteremia due to S pneumoniae.13–17 The roll-plate method is a semiquantitative technique first described by Maki and colleagues in 1977.4 It has been extensively used since that time. A colony count ⬎ 15 on blood agar plate is considered significant and indicates that the catheter is the source of bacteremia. In this case, there were ⬎ 300 cfu on the tip of triple-lumen catheter. This would argue against secondary colonization of the catheter tip from primary bacteremia. Most of the catheter-related bacteremia involves organisms normally found on the skin surface, Staphylococcus aureus being the most serious pathogen.18 Maximum barrier precautions were used at the time of catheter insertion. It was unlikely that the catheter was contaminated by the operator’s nasopharyngeal flora. Pneumococcus is a part of the nasopharyngeal flora. However, it has been suggested that pneumococcus can become a part of the vaginal flora transiently when the organisms are transferred from the upper respiratory tract because of inadequate hygiene. This can cause peritonitis as a result of ascending infection through the female genital tract.19 –20 Bartholin gland infection with S pneumoniae has been reported.19 It is possible that the patient’s right femoral region was colonized with pneumococcus from either the upper respiratory tract or the genital tract. Although uncommon, S pneumoniae can colonize a central venous catheter and can result in bacteremia.

7 8

9 10 11 12 13

14

15 16 17 18 19 20

single-lumen central venous catheters: a prospective study in a critically ill population. Arch Intern Med 1989; 149:1139 – 1143 Benezra D, Kiehn TE, Gold JW, et al. Prospective study of infections in indwelling central venous catheters using quantitative blood cultures. Am J Med 1988; 85:495– 498 Richet H, Hubert B, Nitemberg G, et al. Prospective multicenter study of vascular-catheter-related complications and risk factors for positive central- catheter cultures in intensive care unit patients. J Clin Microbiol 1990; 28:2520 –2525 Tacconelli E, Tumbarello M, Pittiruti M, et al. Central venous catheter-related sepsis in a cohort of 366 hospitalized patients. Eur J Clin Microbiol Infect Dis 1997; 16:203–209 Brun-Buisson C, Abrouk F, Legrand P, et al. Diagnosis of central venous catheter-related sepsis: critical level of quantitative tip cultures. Arch Intern Med 1987; 147:873– 877 Aufwerber E, Ringertz S, Ransjo¨ U. Routine semiquantitative cultures and central venous catheter-related bacteremia. APMIS 1991; 99:627– 630 Groeger JS, Lucas AB, Thaler HT, et al. Infectious morbidity associated with long-term use of venous access devices in patients with cancer. Ann Intern Med 1993; 119:1168 –1174 Armstrong CW, Mayhall CG, Miller KB, et al. Prospective study of catheter replacement and other risk factors for infection of hyperalimentation catheters. J Infect Dis 1986; 154:808 – 816 Pettigrew RA, Lang SD, Haydock DA, et al. Catheter-related sepsis in patients on intravenous nutrition: a prospective study of quantitative catheter cultures and guidewire changes for suspected sepsis. Br J Surg 1985; 72:52–55 Hansell DT, Park R, Jensen R, et al. Clinical significance and etiology of infected catheters used for total parenteral nutrition. Surg Gynecol Obstet 1986; 163:469 – 474 Pemberton LB, Lyman B, Lander V, et al. Sepsis from triplevs single-lumen catheters during total parenteral nutrition in surgical or critically ill patients. Arch Surg 1986; 121:591–594 Ryan JA Jr, Abel RM, Abbott WM, et al. Catheter complications in total parenteral nutrition: a prospective study of 200 consecutive patients N Engl J Med 1974; 290:757–761 Watanakunakorn C, Baird IM. Staphylococcus aureus bacteremia and endocarditis associated with a removable intravenous device. Am J Med 1977; 63:253–256 Westh H, Skibsted L, Korner B. Streptococcus pneumoniae infections of the female genital tract and in the newborn child. Rev Infect Dis 1990; 12:416 – 422 Sirotnak AP, Eppes SC, Klein JD. Tuboovarian abscess and peritonitis caused by Streptococcus pneumoniae serotype 1 in young girls. Clin Infect Dis 1996; 22:993–996

References 1 Musher DM. Infections caused by Streptococcus pneumoniae: clinical spectrum, pathogenesis, immunity, and treatment. Clin Infect Dis 1992; 14:801– 807 2 Watanakunakorn C, Greifenstein A, Stroh K, et al. Pneumococcal bacteremia in three community teaching hospitals from 1980 to 1989. Chest 1993; 103:1152–1156 3 Ruoff KL. Streptococcus. In: Murray PR, Baron MJ, Pfaller MA, et al, eds. Manual of clinical microbiology. 6th ed. Washington, DC: American Society of Microbiology, 1995; 299 –307 4 Maki DG, Weise CE, Sarafin HW. A semiquantitative culture method for identifying intravenous-catheter-related infection. N Engl J Med 1977; 296:1305–1309 5 Fry DE, Fry RV, Borzotta AP. Nosocomial blood-borne infection secondary to intravascular devices. Am J Surg 1994; 167:268 –272 6 Gil RT, Kruse JA, Thill-Baharozian MC, et al. Triple- vs 1516

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

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 Selected Reports

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. (CHEST 2000; 117:1516 –1518) Key words: carcinoma of breast; chemotherapy; neoplasm; yellow nail Abbreviation: YNS ⫽ yellow nail syndrome

nail syndrome (YNS) is a rare disorder, characY ellow terized by rhinosinusitis, pleural effusions, bronchiec-

tasis, 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.

Figure 1. Top a: Dystrophic yellow nails with ridging and loss of lunula. Bottom b: Resolution of yellow nails. Notice absence of lunula in normal nails.

Case Report 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 *From the Division of Pulmonary and Critical Care Medicine, Long Island Jewish Medical Center, New Hyde Park, NY. Manuscript received July 20, 1999; revision accepted October 18, 1999. 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

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 ⫻ 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.

Discussion 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 dystroCHEST / 117 / 5 / MAY, 2000

1517

Figure 2. CT scan demonstrating cylindrical bronchiectasis without pleural effusions.

phic 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.

References 1 Norkild P, Kroman-Anderson H, Struve-Christensen E. Yellow nail syndrome: the triad of yellow nails, lymphedema and pleural effusion. Acta Med Scand 1986; 219:221–227 2 Varney VA, Cumberworth V, Sudderic R, et al. Rhinitis, sinusitis and the yellow nail syndrome: a review of symptoms and response to treatment in 17 patients. Clin Otolaryngol 1994; 19:237–240 1518

3 Emerson PA. Yellow nails, lymphedema and pleural effusion. Thorax 1966;21:247–253 4 Pavlidakey GP, Hashimoto K, Blum D. Yellow nail syndrome. J Am Acad Dermatol 1984; 11:509 –512 5 Gupta AK, Davies GM, Haberman HF. Yellow nail syndrome. Cutis 1986; 37:371–374 6 Guin JD, Elleman JH. Yellow nail syndrome: possible association with malignancy. Arch Dermatol 1979; 115:734 –735 7 Thomas PS, Sidhu B. Yellow nail syndrome and bronchial carcinoma [letter]. Chest 1987; 92:191 8 Mambretti-Zumwalt J, Seidman JA, Higano N. Yellow nail syndrome: complete triad with pleural protein turnover studies. South Med J 1980; 73:995–997 9 Burrows NP, Jones RR. Yellow nail syndrome in association with carcinoma of gall bladder. Clin Exp Dermatol 1991; 16:471– 473 10 Hiller E, Rosenow EC, Olsen AM. Pulmonary manifestations of the yellow nail syndrome. Chest 1972; 61:452– 458 11 Emerson PA. Yellow nails, lymphedema and pleural effusions. Thorax 1966; 21:247–253 12 Siegelman SS, Heckman BH, Hasson J. Lymphedema, pleural effusions and yellow nails: associated immunologic deficiency. Dis Chest 1969; 56:114 –117 13 Stosiek N, Peters KP, Hiller D, et al. Yellow nail syndrome in a patient with mycosis fungoides. J Am Acad Dermatol 1993; 28:792–794 14 Hershko A, Hirshberg B, Nahir M, et al. Yellow nail syndrome. Postgrad Med J 1997; 73:466 – 468 15 Samman PD, White WF. The ’yellow nail syndrome.’ Br J Dermatol 1964; 76:153–157 16 Ilchyshyn A, Vickers CH. Yellow nail syndrome associated with penicillamine therapy. Acta Derm Venereol 1983; 63: 554 –555 17 Runyon BA, Forker EL, Sopko GA. Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails. Am Rev Respir Dis 1979; 119:821– 825 18 Wiggins J, Strickland B, Chung KF. Detection of bronchiectasis by high-resolution computed tomography in the yellow nail syndrome. Clin Radiol 1991; 43:377–379 19 Guin JD, Elleman JH. Yellow nail syndrome: possible association with malignancy. Arch Dermatol 1979; 115:734 –735

Myxoid Liposarcoma of the Supraclavicular Fossa* Michael A. Morse, MD; Edward Bossen, MD; Thomas A. D’Amico, MD, FCCP; Warren Williamson, MD; and Richard Johnson, MD

Liposarcomas generally originate most often in the extremities or retroperitoneum, less frequently in the head and neck, and rarely in the thorax. We describe a particularly rare presentation of myxoid liposarcoma originating in the supraclavicular fossa. The mass was resected and has not recurred. We searched our pathology database for other softtissue tumors of the supraclavicular fossa and found no other case of sarcoma originating in this site. In addition, we performed a literature review of thoracic and neck liposarcomas to identify similar cases and discuss their clinical course. (CHEST 2000; 117:1518 –1520) Key words: myxoid liposarcoma; soft-tissue sarcomas; supraclavicular fossa Selected Reports