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Year : 2013  |  Volume : 30  |  Issue : 2  |  Page : 164-165  

Sudden onset of dyspnea in a woman with skin lesions and lung cysts

1 Department Pulmonary and Critical Care Medicine, Dayanand Medical College and Hospital, Ludhiana, India
2 Department of Anesthesiology, Peninsula Regional Medical Center, Salisbury MD, USA

Date of Web Publication11-Apr-2013

Correspondence Address:
Akashdeep Singh
Department Pulmonary and Critical Care Medicine, Dayanand Medical College and Hospital, Ludhiana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-2113.110433

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How to cite this article:
Singh A, Singh J. Sudden onset of dyspnea in a woman with skin lesions and lung cysts. Lung India 2013;30:164-5

How to cite this URL:
Singh A, Singh J. Sudden onset of dyspnea in a woman with skin lesions and lung cysts. Lung India [serial online] 2013 [cited 2020 Jun 3];30:164-5. Available from: http://www.lungindia.com/text.asp?2013/30/2/164/110433

A 40-year-old female presented with complaints of sudden onset left sided chest pain, dry cough, and shortness of breath of one day duration.

On admission she was anxious, cyanosed, dyspnoeic and had obvious respiratory distress. She had an oxygen saturation of 78% while breathing room air, respiratory rate of 30/minutes, blood pressure of 140/90 mmHg, and a pulse of 126/minute. Respiratory examination revealed decreased movements, hyperresonance on percussion and distant lung sounds involving the entire left hemithorax. There were multiple, thick, fibrous, reddish-brown papules around the nose, cheeks, and chin conglomerulating to form a characteristic butterfly-shaped pattern [Figure 1]a. Besides this, there were fibromatous plaques over forehead and scalp [Figure 1]a. Her back revealed multiple ash leaf shaped hypopigmented macules and toenails revealed periungual fibromas [Figure 1]b. Her past history revealed that she had mild learning difficulty and had studied till fifth standard and was un-married.
Figure 1:

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Laboratory investigations including hemogram and routine biochemistry were normal. Chest radiograph showed hypertranslucent left hemithorax [Figure 2]a. Computed tomography of chest revealed left sided pneumothorax and moderate sized right pleural effusion [Figure 2]b. The lung parenchyma revealed bilateral, multiple, variable sized cysts [Figure 2]b with normal intervening lung and preserved volumes. Diagnostic pleurocentesis revealed milky fluid, which was exudative lymphocytic predominant with pleural fluid proteins of 4g/dl, sugar of 100mg/dl, LDH of 344 U/L, pH of 7.60, triglycerides of 130 mg/dl, and cholesterol of 30 mg/dl. Abdominal ultrasound revealed bilateral multiple angiomyolipomas in the kidney and liver.
Figure 2:

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   Question Top

Q 1: What is your diagnosis?

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   References Top

1.Johnson S. Lymphangioleiomyomatosis: Clinical features, management and basic mechanisms. Thorax 1999;54:254-64.  Back to cited text no. 1
2.Franz DN, Brody A, Meyer C, Leonard J, Chuck G, Dabora S, et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001;164:661-8.  Back to cited text no. 2
3.Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 2010;35:14-26.  Back to cited text no. 3
4.Hancock E, Tomkins S, Sampson J, Osborne J. Lymphangioleiomyomatosis and tuberous sclerosis. Respir Med 2002;96:7-13.  Back to cited text no. 4


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