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December 1957

Dyskeratosis Congenita: Relationship to Poikiloderma Atrophicans Vasculare and to Aplastic Anemia of Fanconi

Author Affiliations


From the Department of Dermatology and Syphilology of Western Reserve University School of Medicine and The University Hospitals of Cleveland.

AMA Arch Derm. 1957;76(6):712-719. doi:10.1001/archderm.1957.01550240030006

There have been between 230 and 250 reports of poikiloderma atrophicans vasculare (Jacobi).

Cockayne quotes Oppenheim to the effect that the disease is familial, but adds, "The evidence for this statement is scanty." (p. 363.)

In June, 1906, Petges and Cléjat reported their case of "sclérose atrophique de la peau et myosite generalisée," and at the Ninth Dermatological Congress at Berne, September, 1906, Jacobi presented his case of poikiloderma atrophicans vasculare. In 1909 Professor Zinsser reported familial cases, two brothers, affected with a disease closely resembling the pictures as described by Jacobi, with the skin manifestations, the dystrophic nails, and the leukoplakia of the mucous membranes.

In the comprehensive report of Oliver on the condition, his patient ended with mycosis fungoides, as did the patient of Weiss and that of Wile. Civatte's patient had been diagnosed as having parapsoriasis by Brocq, as having poikiloderma atrophicans vasculare by