CASE REPORT

The Clinical Respiratory Journal

Yellow nail syndrome presenting with postpartum massive pleural effusion Hülya Günbatar1, Bunyamin Sertogullarindan1, Selami Ekin1, Ahmet Arisoy2 and Hatice Uce Özkol3 1 Department of Pulmonary and Critical Care, Yuzuncu Yil University Medical Faculty, Van, Turkey 2 Private Istanbul Hospital, Van, Turkey 3 Department of Dermatology, Yuzuncu Yil University Dursun Odabas Tip Merkezi, Van, Turkey

Abstract Yellow nail syndrome (YNS) is an uncommon clinical syndrome characterized by yellow-green discoloration of nails and recurrent respiratory tract lesions, pleural effusions and lymphedema. A 38-year-old woman was referred to the emergency complaining of chronic cough and increasing dyspnea within last 2 weeks. She had given birth 1 month ago. On examination, the patient exhibited dystrophic yellowish nails and mild peripheral lymphedema. A chest roentgenogram revealed a large right pleural effusion and a small left pleural effusion. YNS is a rare clinical entity but should be taken into consideration in patients with massive pleural effusions and persistent lymphedemas on the postpartum period. Please cite this paper as: Günbatar H, Sertogullarindan B, Ekin S, Arisoy A and Özkol HU. Yellow nail syndrome presenting with postpartum massive pleural effusion. Clin Respir J 2014; ••: ••–••. DOI:10.1111/crj.12077.

Conflict of interest The authors have stated explicitly that there are no conflicts of interest in connection with this article.

Introduction The diagnosis of yellow nail syndrome (YNS) should be considered in a patient with two of the three findings associated with the disorder, after other possible causes of the abovementioned symptoms are excluded (1, 2). The respiratory illness component is believed to be due to hypoplasia of the lymphatic system or an immunological deficiency (3). Attempted treatment is most often aimed at alleviating symptoms (2). The presented report describes a patient with YNS and massive pleural effusion after giving birth.

Case presentation A 38-year-old woman was referred to the emergency clinic of Yuzuncu Yil University (Van, Turkey) com-

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2013 John Wiley & Sons Ltd

Key words massive – pleural effusion – postpartum – yellow nail syndrome Correspondence Hülya Günbatar, MD, Y.Y.U. D. Odabas Tip Merkezi Kampüs, Van, Turkey. Tel: +90432 2150470-6135 Fax: +90 5065118827 email: [email protected] Received: 15 August 2013 Revision requested: 30 October 2013 Accepted: 20 November 2013 DOI:10.1111/crj.12077 Authorship and contributorship Dr. Günbatar, Dr. Sertogullarindan and Dr. Ekin contributed in the follow-up and treatment of patients, and in designing and writing the manuscript. Dr. Özkol contributed in writing the dermatological features of yellow nail syndrome this article. Dr. Arisoy contributed in designing and writing the manuscript.

plaining of chronic cough and increasing dyspnea within the last 2 weeks. She had given birth 1 month ago; she had first noticed the symptoms approximately 2 months before presentation. The patient’s history revealed recurrent infections of the respiratory tract. There was no history of smoking and allergies. She has had nail changes since childhood (Fig. 1). She had edema of her lower extremities for 2 months. On examination, the patient exhibited dystrophic yellowish nails and mild peripheral lymphedema. The lower half of her right chest was dull to perfusion, and there were no breathe sounds over this area. The results of the following laboratory studies were negative or normal: complete blood count, urinalysis, serum creatinine, serum electrolytes, cholesterol, procalcitonin, thyroid function tests and electrocardiogram. Sputum cultures were negative. A chest roentgenogram

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tube drainage. She received amoxicillin and diuretic for 7 days, chest tube was remowed. The patient was discharged from the hospital after 20 days. One month later, she had marked thickening of bilaterally but no free fluid was demonstrated and no further thoracentesis has been required.

Discussion

Figure 1. Bilaterally pleural effusions, atelectasis in the right lung on computed tomography of the chest.

revealed a large right pleural effusion and a small left pleural effusion. Computed tomography of the chest revealed bilaterally pleural effusions, atelectasis in the right lung (Fig. 2). There were no pulmonary embolism and consolidation. Studies of fluid showed that it was an exudate with albumin 2.02 g/dL, total protein 3.43 g/dL, lactate dehydrogenase 335 U/L, glucose 90 mg/dL and 50% lymphocytosis. Cytologic examination of the fluid for malignant cells and culture were negative. Tuberculosis culture in the sputum and pleural liquid, adenosine deaminase (16 IU/L) were negative. Echocardiography revealed normal cardiac function. On the following days, her shortness of breath worsened and new chest roentgenogram showed massive right pleural effusion. The right lung expanded with

Figure 2. The nail yellowish changes of patient.

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YNS is an uncommon clinical syndrome characterized by yellow-green discoloration of nails and the presence of respiratory tract lesions, pleural effusions and lymphedema with onset generally after puberty (1, 2). Causes of YNS remain unknown; however, some suspect that it occurs secondary to conditions such as connective tissue disease, thyroid disorder or in some cases an adverse effect to drugs such as penicillamine and bucillamine (3). The pathogenesis of YNS is unclear; it is believed that the syndrome may be due to a structural and functional dysfunction of the lymphatic system coupled with an increased vascular permeability to albumin resulting in the observed lymphedema and yellow nails (4). Spontaneous remissions were also observed (5). YNS mainly affects middle-aged persons, but some cases were described in children or the elderly (6, 7). The disease is seen more commonly in women, with a female/male ratio of 1.6:1 (1, 7). Considering the triad of yellow dystrophic nails, lymphedema and pleural effusion with recurrent lower respiratory tract infections, YNS was diagnosed. YNS is characterized by slowly growing, dystrophic yellow nails, peripheral lymphedema, pleural effusions, rhinosinusitis and bronchiectasis with consecutive recurrent lower respiratory tract infections (1, 2). Individual manifestations of the syndrome can appear at different times, and clinical onset varies from birth to late adult life (8). Symptoms and signs from the respiratory tract were added to the classical clinical picture of YNS, including chronic paranasal sinusitis, recurrent respiratory tract infections and bronchiectases. In the present case, there was bilaterally pleural effusion, yellow nails and recurrent lower respiratory tract infections. A pleural effusion without apparent cause usually is labeled ‘idiopathic’ pleural effusion. The cause of the pleural effusion entity is most likely the same as the cause of the lymphedema and yellow nails, underdeveloped hypoplastic lymphatics. The precipitation event is unknown but lymphedema frequently became clinically evident after injury, insect bites or an infection such as erysipelas which increased the load on

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2013 John Wiley & Sons Ltd

Günbatar et al.

the already deficient lymphatics. Pregnancy could be added these factors. Venous congestion and relative lymphatic obstruction is the main cause of generalized edema seen during pregnancy; hypoplastic lymphatics may aggravate the condition. Pleural effusion was the first ever described abnormality in YNS. Effusion is most often of exudate type and contains mostly lymphocytes, and the lactate dehydrogenase level is increased. High albumin content in effusion can be related to protein leakage at the capillary level, as suggested by some authors (4). In our case, there was lymphocytes predominantly exudative pleural effusion. Respiratory tract infections were also predominant features in the case reports of other authors (9, 10). The frequent findings of sinusitis may offer some explanation of bronchiectasis. Hurwitz and Pinals believed that both patients had an immunologic deficiency state: they demonstrate leukopenia, lymphopenia and a significant reduction of y-globulin in the male siblings (11). In the presented patient, there was no bronchiectasis; if followed for a long time, bronchiectasis could be developed in our case. The diagnosis is made clinically, and no specific treatment is known. The prognosis of YNS is generally good, and supportive measures such as rotating antibiotic therapy, thoracentesis and corticosteroid therapy are often helpful in controlling the symptoms of the disease (12). Spontaneous remissions were noted in some patients (13). In a YNS with glomerulonephritis patient, immunosuppressive treatment had no impact (14). The greatest clinical concerns are however related to recurrent pleural effusions, as pleural cavity drainage gives only short-term improvement in most cases; pleurodesis appears to be more effective. Some authors successfully administered bleomycin, tetracycline and OK-432 (15, 16). Pleuro-peritoneal shunts can be implanted, with alternative shunts between the venous system and the pleural or peritoneal cavity (2, 16). YNS is a rare clinical entity, but it should be taken into consideration in patients with massive pleural effusions and persistent lymphedemas on the postpartum period as shown in this case.

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2013 John Wiley & Sons Ltd

Massive pleural effusion with yellow nail syndrome

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Yellow nail syndrome presenting with postpartum massive pleural effusion.

Yellow nail syndrome (YNS) is an uncommon clinical syndrome characterized by yellow-green discoloration of nails and recurrent respiratory tract lesio...
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