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Dysplastic nevus syndrome

A dysplastic nevus, (or naevus; pl. nevi or naevi) is an atypical mole; a mole whose appearance is different from that of common moles. Dysplastic nevi are generally larger than ordinary moles and have irregular and indistinct borders. Their color frequently is not uniform and ranges from pink to dark brown; they usually are flat, but parts may be raised above the skin surface. more...

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Dysplastic nevus syndrome


According to the National Cancer Institute, doctors believe that dysplastic nevi are more likely than ordinary moles to develop into a type of skin cancer called melanoma. Because of this, moles should be checked regularly by a doctor or nurse specialist, especially if they look unusual; grow larger; or change in color, outline, or in any other way.


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Hemothorax and hemopericardium in a patient with Bean's blue rubber bleb nevus syndrome
From CHEST, 6/1/89 by David Langleben

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

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

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


The patient is a 58-year-old retired textile cutter of Italian origin who smokes cigarettes and denies asbestos exposure. In 1968, he had presented 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 removed 1,100 ml of serosanguinous fluid. The tamponade recurred, requiring another pericardiocentesis. A left thoracocentesis extracted 700 ml of serosanguinous fluid containing fibrin, hemolyzed red cells, lymphocytes, and mesothelial cells. Cultures 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 on diuretics for the next 18 years. He was asymptomatic and working full time.

In March 1986, he presented to another hospital with a one-month history of dyspnea and a right sided pleural effusion. Thoracocentesis extracted bloody fluid which did not contain malignant cells 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 removed serosanguineous fluid containing only blood cells. A right thoracotomy was performed, 1,000 ml of bloody fluid was removed, and the right lung was found 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 a loose connective tissue stroma attached to the visceral pleura with a short stalk (Fig 1). The endothelial cells were either flat or slightly swollen, but possessed no atypical cytologic features. Except for slight fibrotic thickening of the pleura, the pleura and lung were otherwise unremarkable. Granulomas and malignant cells were absent. No specific therapy was administered and ankle swelling has persisted despite furosemide therapy. The pleural effusion has not recurred and a recent cardiac catheterization suggested mild pericardial constriction.

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


The term "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 upon 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 over 40 cases reported. Tissues and organs affected include the liver, spleen, 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] but pleural hemorrhage has not previously been described.

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

We do not know if our patient has pericardial involvement, although angiomas of the pericardium have been described in the blue rubber bleb nevus syndrome.[10] The presence of angiomas in the pericardium might predispose to bleeding spontaneously or from a viral pericarditis. In this regard, it is interesting that our patient had a significant rise in titers for coxsackie A9 virus. However, this agent is not known to affect either pericardium or pleura.[11] More likely, therefore, the pericardial bleed represented a spontaneous event in a patient with pericardial angiomas. Aside from gastrointestinal, nasal, and genitourinary bleeding, hemorrhage from other sites has not previously been described in the blue rubber bleb nevus syndrome. We have shown that intrathoracic bleeding can occur in this syndrome and may pose life-threatening problems. Although the syndrome is uncommon, it may often be recognized by a careful skin examination and should be familiar to all internists who diagnose and manage pleural and pericardial effusions.

ACKNOWLEDGMENTS: We thank Christina Tsioutsias for preparation of the manuscript.

([unkeyable]) Scholar of the Canadian Heart Foundation.


1 Bean WB. Vascular spiders and related lesions of the skin. Springfield, IL: Charles C Thomas, 1958:178-85

2 Fretzin DF, Potter B. Blue rubber bleb nevus. Arch Intern Med 1965; 116:924-29

3 Berlyne GM, Berlyne N. Anaemia due to blue rubber bleb naevus disease. Lancet 1960; 2:1275-77

4 Morris SJ, Kaplan SR, Ballan K, Tedesco FJ. Blue rubber bleb nevus syndrome. JAMA 1978; 239:1887

5 Rice JS, Fischer DS. Blue rubber bleb nevus syndrome. Arch Dermatol 1962; 86:503-11

6 Wood MW, White RJ, Kernohan JW. Cancerous hemangiomatosis involving the brain, spinal cord, heart, skin and kidney. Staff Meetings of Mayo Clin 1957; 32:249-54

7 Crepeau J, Poliquin J. The blue rubber bleb nevus syndrome. J Otolarnygol 1981; 10:387-90

8 Belsheim MR, Sullivan SN. Blue rubber bleb nevus syndrome. Canad J Surg 1980; 23:274-75

9 Smart RH, Newton DE. Hemangioma of the penis with blue rubber bleb syndrome. J Urol 1975; 113:570-71

10 Waybright EA, Selhorst JB, Rosenblum WI, Suter CG. Blue rubber bleb nevus syndrome with CNS involvement and thrombosis of a vein of Galen malformation. Ann Neurol 1978; 3:464-67

11 Melnick JL. Enteroviruses: polioviruses, coxsackieviruses, echoviruses, and newer enteroviruses. In: Fields BN, ed. Virology. New York: Raven Pess, 1985:756-57

COPYRIGHT 1989 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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