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Progressive Dyspnea in a Woman With Genital Skin Lesions

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

An 82-year-old woman presented with dyspnea on exertion and weight gain of 5 kg over the past month. Her medical history included atrial fibrillation and lung adenocarcinoma (a lung lobectomy was performed 8 years ago). Her blood pressure was 112/60 mm Hg, her heart rate was 100 beats per minute, her respiratory rate was 37 breaths per minute, her body temperature was 36.6°C, and her oxygen saturation was 100% while breathing 10 L of oxygen. A cardiac examination was clinically significant for an irregular rhythm, an increased pulmonic closure sound, and jugular venous distension. Bibasilar coarse crackles and pretibial edema were also noted. A complete blood count had results within normal limits. The serum lactate dehydrogenase level was 278 U/L (to convert to microkatal per liter, multiply by 0.0167). A chest radiographic image showed bibasilar infiltrates. An electrocardiogram was notable for atrial fibrillation, and echocardiography revealed normal left ventricular function, mild right atrial and ventricular dilatation, and severe tricuspid regurgitation (transtricuspid pressure gradient, 40 mm Hg). A contrast-enhanced computed tomography image revealed bilateral ground-glass opacities and right basilar consolidations, with no evidence of thromboembolisms (Figure, A). Para-aortic and inguinal lymphadenopathies were also noted. Presumptive diagnoses of pulmonary hypertension (PH) and right-sided heart failure were made. The patient began receiving dobutamine and furosemide, but she continuously required 2 to 10 L of oxygen, and further invasive evaluations could not be performed. An echocardiogram identified aggravating PH (transtricuspid pressure gradient, 57 mm Hg). A thorough physical examination identified scaly, crusty genital skin lesions pathologically compatible with extramammary Paget disease. The patient deteriorated and died despite treatment. An autopsy was performed (Figure, B).

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C. Pulmonary tumor thrombotic microangiopathy

The salient features of this case are the subacute exacerbation of right-sided heart failure and PH, along with ground-glass opacities on computed tomography and genital skin lesions. Pulmonary thromboembolism must be considered, but no evidence of thromboembolism was confirmed on imaging. With this in mind, clinicians must consider alternative causative mechanisms for worsening right-sided heart failure and PH, such as pulmonary tumor thrombotic microangiopathy (PTTM) and acute exacerbation of chronic thromboembolic pulmonary hypertension. For this patient, PTTM is the leading hypothesis, given her skin lesions, lymphadenopathies, and pathological findings compatible with extramammary Paget disease. No evidence of left ventricular systolic dysfunction was obtained by ultrasonography. Although sarcoidosis can cause PH, her medical history and pathology results were incompatible with this diagnosis.

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Article Information

Corresponding Author: Hiroyuki Teruya, MD, Department of Internal Medicine, Okinawa Chubu Hospital, 281 Miyazato, Uruma, Okinawa 9042293, Japan (hiroyukiyuki0221@gmail.com).

Published Online: January 9, 2020. doi:10.1001/jamaoncol.2019.5863

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s family member for granting permission to share the patient’s clinical information. We also thank Mitsuyo Kinjo, MD, MPH, and Hiroyuki Yano, MD, Department of Internal Medicine, Okinawa Chubu Hospital, for their thoughtful comments on the earlier version of this manuscript. The named individuals were not compensated for their contributions.

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