David Langleben, M.D.;([unkeyable])...

David Langleben, M.D.;([unkeyable]) Norman Wolkove MD FCCP; Herbert Srolovitz, MD; Robin C Billick, MD; and Nathan M Sheiner, MD

A patient with Bean's sapphirine rubber bleb nevus syndrome at handed with a hemothorax, and 18 years earlier had readyed with "idiopathic" hemopercardium and tamponade. Typical hemangiomas were build in the pleura and skin. This is the first report of intrathoracic bleeding with this disorder.

In 1958 Bean[1] described rubbery azure hemangiomas of the skin and gastrointestinal tract associated with gastrointestinal bleeding, which he seasoned the "blue rubber bleb nevus syndrome" following papers have identified hemangiomas in other visceral organs, including the pleura. Pleural bleeding, however, has not been reported. We now describe a patient who existinged with a hemothorax and had the classic skin findings of gloomy rubber bleb nevus syndrome and hemangiomas of the pleura. This patient had also bring to maturityed a hemopericardium 18 years prior to the hemothorax, possibly related to the same disease process

CASE REPORT

The patient is a 58-year-old retired textile cutter of Italian origin who idle talks cigarettes and denies asbestos front In 1968, he had not awayed to another hospital in cardiac tamponade, with a two-week history of orthopnea and leg edema. The chest roentgenogram revealed an enlarged globular cardiopericardial silhouette and a density at the left base suggestive of effusion. Pericardiocentesis remov 1100 ml of serosanguinous fluid. The tamponade recurr requiring another pericardiocentesis. A left thoracocentesis extracted 700 ml of serosanguinous fluid containing fibrin, hemolyzed r solitary abode; squalids lymphocytes, and mesothelial cells. cultivations of both fluids were negative. There was no clinical or laboratory evidence for malignancy, tuberculosis, or autoimmune disease. Acute and convalescent viral titers for coxsackie A9 were 1/24 and 1/768 and for coxsackie B4 1/48 and 1/64 The patient was treated with prednisone and then continued onward diuretics for the next 18 years. He was asymptomatic and working well stocked [i]or[/i] provided time.

In March 1986 he neared to another hospital with a one-month history of dyspnea and a right sided pleural effusion. Thoracocentesis extracted murderous fluid which did not contain malignant small cavitys or Mycobacteria and which increased despite furosemide therapy.

In July 1986 he was seen at our hospital where vital signs were normal. The chest roentgenogram showed right-sided pleural fluid and marked pleural thickening. Thoracocentesis remov serosanguineous fluid containing merely blood cells. A right thoracotomy was performed, 1000 ml of gory fluid was removed, and the right lung was fix to be surrounded by a thickened fibrous parietal pleura and was trapped within a similarly thickened visceral pleura. A pleural and superficial lung biopsy showed vascular lesions (capillary and arteriolar-venular type) arranged in a lobular pattern, supported by dint of a loose connective tissue stroma attached to the visceral pleura with a short stalk (Fig 1) The endothelial small cavitys were either flat or slightly swollen however possessed no atypical cytologic features. exclude for slight fibrotic thickening of the pleura, the pleura and lung were otherwise unremarkable. Granulomas and malignant small cavitys were absent. No specific therapy was administered and ankle swelling has persisted despite furosemide therapy. The pleural effusion has not recurr and a novel cardiac catheterization suggested mild pericardial constriction.

in succession a recent visit, multiple violaceous compressible venous lakes were recognized throughout the arms, chest, and back, and the patient said these were a lifelong finding. The diagnosis of pallid rubber bleb nevus syndrome was considered and a skin biopsy was performed. It showed an attenuated epidermis in which a cavernous hemangioma was at hand (Fig 2). The vascular walls were thin, endothelial lonely dwellings typical, and several fibrin thrombi were present

DISCUSSION

The period of time "blue-rubber-bleb-nevus," first coined by Bean,[1] applies to a syndrome of rubbery angiomas of the skin, variable in size and number which are compressible and refill immediately after release of the compression. Associated with this cutaneous abnormality, angiomas of the GI tract have been reported to cause profuse bleeding.[1-4] Although rare, the syndrome has been recognized in the literature and above 40 cases reported. Tissues and organs affected include the liver, dejection central nervous system, skeletal muscle, heart, oro- and nasopharnyx, adrenal glands, kidney, thyroid, and glans penis.[3-9] Pleural and lung involvement has been mentioned,[1-5] nevertheless pleural hemorrhage has not previously been described.

Our patient currented with a hemothorax, had pleural angiomas finded by histologic study, and had typical skin findings. Infectious and autoimmune etiologies for the pleural effusion were exclud It is likely that the bleeding angioma caused the pleural effusion.