Lower extremity edema is a rarely reported presentation of sarcoidosis. We recently encountered a patient with upper extremity swelling as the initial feature of pulmonary sarcoid.
A 53-year-old female presented with three weeks of symmetrical bilateral upper extremity swelling. She denied pain or trauma involving the arms, respiratory symptoms, history of coagulopathy and systemic complaints including fever, weight loss or night sweats. Her past medical history was significant for ductal carcinoma in situ of the right breast, for which she underwent lumpectomy. Serial mammograms revealed no evidence of recurrence. Current medications included Tamoxifen and vitamins. Her physical exam confirmed swollen upper extremities, which was most pronounced in the forearms. The neuromuscular, vascular, pulmonary and skin exams were normal. No facial swelling or adenopathy was appreciated. Routine laboratories were normal except for a mild anemia. A venous duplex scan demonstrated non-obstructed flow of the upper extremities. A chest radiograph suggested hilar and mediastinal adenopathy, which was confirmed on contrast enhanced chest CT. (figure 1). No parenchymal lung disease or other abnormalities were detected and the superior vena cava (SVC) was patent. A mammogram was unremarkable. Malignancy, infection or an inflammatory process, such as sarcodosis, leading to adenopathy and lymphatic obstruction, were considered potential etiologies of the patient’s physical exam and radiographic findings. Mantoux test was non-reactive and pulmonary function tests were normal. An angiotensin converting enzyme inhibitor level was slightly elevated at 59 U/L. A flexible fiberoptic bronchoscopy was performed and samples were sent for flow cytometry, microbiology, cytology and histology. A transbronchial biopsy sample showed non-caseating granulomas with lymphocytic cuffing suggestive of sarcoidosis. Bacterial, mycobacterial and fungal stains were negative. Based on these results, the patient was placed on oral steroids (Prednisone 40mg qd) for a presumptive diagnosis of Stage 1 pulmonary sarcoidosis. A follow up evaluation at 14 weeks showed significant resolution of the upper extremities swelling. A repeat chest CT scan after 3 months of treatment demonstrated decreased hilar and mediastinal lymphadenopathy. (figure 2).
Involvement of the extremities in sarcoidosis frequently includes skin and musculoskeletal abnormalities, although a number of atypical presentations have been described. These include tumorous sarcoid myopathy causing unilateral painless swelling of an upper extremity, bilateral leg lymphedema attributed to abdominal and inguinal adenopathy and pain, erythema and swelling of the legs. Superior vena cava syndrome has also been reported. Isolated upper extremity edema is rare, and frequently is due to malignant SVC obstruction or thrombosis. In our patient these entities were excluded, and we suspect that her upper extremity edema was caused by lymph flow blockage from systemic lymph node involvement of sarcoid. As in cases involving lower extremity lymphedema, she responded well to steriod therapy. To our knowledge, this is the first reported case of sarcoidosis presenting with isolated upper extremity edema.
Sarcoidosis is a potential cause of extremity lymphedema and may be the initial presentation of the disease.
S.M. Zeineldine, None.