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October 1957

Fatal Scleroderma with L. E. Phenomenon: Report of a Case

Author Affiliations

Honolulu, Hawaii

AMA Arch Derm. 1957;76(4):427-430. doi:10.1001/archderm.1957.01550220035006

The L. E. factor is not found exclusively in systemic lupus erythematosus. It has been reported in hemolytic anemia, multiple myeloma, leukemia, miliary tuberculosis, pernicious anemia, dermatitis herpetiformis, drug reactions to penicillin and hydralazine (Apresoline), diffuse systemic rheumatic disease, and diffuse progressive scleroderma.1

Systemic lupus erythematosus is a protean disease, however, and may be manifested only by a single finding, such as fever, or albuminuria, during a part of its course. In many of the above instances it is difficult, and in a few it is impossible, to be sure that the obvious disease was not a manifestation of, or accompanied by, unrecognized systemic lupus erythematosus.

The present report concerns a case of typical diffuse progressive scleroderma in which, during nearly a year's close observation (including autopsy) not a single finding occurred to warrant even a suspicion that the disease was accompanied by systemic