Progressive Dyspnea in a Woman With Genital Skin Lesions
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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

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.

Because PTTM has an almost complete case-fatality rate, the treatment of underlying malignant conditions is the cornerstone of management. Several case reports identified imatinib as a treatment option to increase the survival rate and alleviate pulmonary hypertension associated with PTTM.This case highlights the importance of considering PTTM as a differential diagnosis in patients presenting with a combination of aggravating dyspnea, PH, and normal pulmonary imaging findings. A high index of suspicion allows clinicians to perform both the treatment of the underlying malignant condition and advanced-care planning appropriately.

Source:https://jamanetwork.com/journals/jamaoncology/article-abstract/2758263
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