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A Syndrome with Characteristic Cutaneous Manifestations

CAPT. HOMER E. WILLIAMS, MC; CAPT. D. JOSEPH DEMIS, MC; COL. ROBERT S. HIGDON, MC

Arch Dermatol. 1960;82(6):937-942.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Cerebellar ataxia with ocular and cutaneous telangiectasia was first described in a 9-year-old Caucasian boy by Madame Louis-Bar¹ in 1941. The next report in which there were similar findings was made in 1957 by Wells and Shy.² These authors described two sisters who at the ages of 5 and 7 years experienced the onset of progressive ataxia followed, approximately 3 years later, by oculocutaneous telangiectasia. In 1957, Biemond³ also reported 4 similar cases, including 2 necropsies. In 1958, Boder and Sedgwick⁴ described 8 cases under the title "Ataxia-Telangiectasia." At approximately the same time 2 additional cases were reported by Centerwall and Miller.⁵ Since 1958 there have been additional sporadic reports^7,9 bringing the total number of known cases of this unusual syndrome to 24.

The syndrome, as described by Boder and Sedgwick, has 4 main features— progressive cerebellar ataxia, progressive oculocutaneous telangiectasia, frequent sinopulmonary infections, . . . [Full Text PDF of this Article]

Author Affiliations
USA; USA; USA

From the Dermatology Service, Walter Reed General Hospital, and the Department of Dermatology, Water Reed Army Institute of Research, Washington, D.C.

Footnotes
Submitted for publication June 30, 1960.

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