FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 329:1237-1239 October 21, 1993 Number 17 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Pseudoxanthoma elasticum is an inherited disorder of connective tissue that is associated with numerous systemic manifestations, including premature coronary artery disease. Without a serologic marker, the diagnosis relies on clinical features and the histologic demonstration of abnormal, calcified elastic fibers^1,2.

The typical patient presents with yellow macules or papules that can become confluent to form plaques and, in severe cases, redundant folds of skin. Cutaneous lesions have been likened to "plucked chicken skin." In patients whose skin is mildly affected, the condition may be difficult to recognize. Ocular complications occasionally occur without the characteristic skin lesions. The histologic diagnosis . . . [Full Text of this Article]

Case Reports

Patient 1

Patient 2

Patient 3

Patient 4

Methods

Results

Discussion

Source Information

From the Departments of Dermatology (M.L., R.G.P.) and Pathology (R.G.P.) and the Division of Cardiology, Department of Internal Medicine (J.H.), Mount Sinai Medical Center, New York.

Address reprint requests to Dr. Lebwohl at Mount Sinai Medical Center, 5 E. 98th St., Box 1048, New York, NY 10029-6574.

References

Related Letters:

Case 43-1993: Occipital Mass
Vertosick F. T., Henson J. W.
Extract | Full Text  
N Engl J Med 1994; 330:793, Mar 17, 1994. Correspondence

This article has been cited by other articles:

• Schon, S, Schulz, V, Prante, C, Hendig, D, Szliska, C, Kuhn, J, Kleesiek, K, Gotting, C (2006). Polymorphisms in the xylosyltransferase genes cause higher serum XT-I activity in patients with pseudoxanthoma elasticum (PXE) and are involved in a severe disease course. J. Med. Genet. 43: 745-749 [Abstract] [Full Text]  
• Hendig, D., Schulz, V., Arndt, M., Szliska, C., Kleesiek, K., Gotting, C. (2006). Role of Serum Fetuin-A, a Major Inhibitor of Systemic Calcification, in Pseudoxanthoma Elasticum. Clin. Chem. 52: 227-234 [Abstract] [Full Text]  
• Chassaing, N, Martin, L, Calvas, P, Le Bert, M, Hovnanian, A (2005). Pseudoxanthoma elasticum: a clinical, pathophysiological and genetic update including 11 novel ABCC6 mutations. J. Med. Genet. 42: 881-892 [Abstract] [Full Text]  
• Song, H. K., Sharoni, E., Williams, B. Jr, Guyton, R. A., Puskas, J. D. (2004). Long-term left internal mammary artery graft patency for coronary artery disease associated with pseudoxanthoma elasticum. Ann. Thorac. Surg. 78: 691-693 [Abstract] [Full Text]  
• Iliopoulos, J., Manganas, C., Jepson, N., Newman, D. C. (2002). Pseudoxanthoma elasticum: is the left internal mammary artery a suitable conduit for coronary artery bypass grafting?. Ann. Thorac. Surg. 73: 652-653 [Abstract] [Full Text]  
• Aessopos, A., Farmakis, D., Loukopoulos, D. (2002). Elastic tissue abnormalities resembling pseudoxanthoma elasticum in beta thalassemia and the sickling syndromes. Blood 99: 30-35 [Abstract] [Full Text]  
• Aessopos, A., Samarkos, M., Voskaridou, E., Papaioannou, D., Tsironi, M., Kavouklis, E., Vaiopoulos, G., Stamatelos, G., Loukopoulos, D. (1998). Arterial Calcifications in {beta}-Thalassemia. ANGIOLOGY 49: 137-143 [Abstract]  
• Kevorkian, J.-P., Masquet, C., Kural-Menasche, S., Le Dref, O., Beaufils, P., Kevorkian, J.-P. (1997). New Report of Severe Coronary Artery Disease in an Eighteen-Year-Old Girl with Pseudoxanthoma Elasticum: Case Report and Review of the Literature. ANGIOLOGY 48: 735-741 [Abstract]  
• Grossi, E. A., Pasternack, P., Lebwohl, M., Phelps, R., Halperin, J. (1994). Pseudoxanthoma Elasticum. NEJM 330: 793-793 [Full Text]