Cutaneous Signs and Syndromes Associated With Internal Malignancies

Claudia C. Ramirez, MD; Brian Berman, MD, PhD


Skinmed. 2005;4(2):84-92. 

In This Article

Superior Vena Cava Syndrome and Werner's Syndrome

The superior vena cava is the major drainage vessel from the head, neck, upper thorax, and upper extremities. Superior vena cava syndrome (SVCS) is caused by a gradual compression of the superior vena cava resulting in obstruction of its blood flow. Dyspnea and facial swelling are the most common symptoms. Facial and upper extremities edema, and jugular venous distention with or without cyanosis are usually present on physical examination. The most common etiology for SVCS is a mediastinal malignancy. Bronchogenic carcinoma constitutes 80% of cases, small cell carcinomas being the majority of these lung cancers, with lymphoma representing 18% of cases.[41] Less commonly, SVCS may be caused by metastases of other solid malignancies. Nonmalignant causes of SVCS include mediastinal fibrosis, aortic aneurysm, vasculitis, infections, benign mediastinal tumors, and thrombosis related to the presence of central vein catheters.

Werner's syndrome is a rare autosomal recessive disease resulting in premature aging. It is characterized by cataracts, short stature, premature graying of the hair and baldness, laryngeal atrophy with high-pitched voice, distal muscle atrophy, and endocrine disorders such as diabetes mellitus, osteoporosis, and hypogonadism. Among the skin manifestations of Werner's syndrome, dryness and atrophy, scleroderma-like appearance, beak-nose, hyperkeratosis over bone prominences, and chronic leg ulcers most often are present.