Degos' disease, known also as malignant atrophic papulosis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system, the lesions in the latter two organs often resulting in death.¹ Our interest in this subject derived from an opportunity to study sections of tissue from a biopsy specimen taken from a 35-year-old man who had skin lesions stereotypical clinically of Degos' disease (Fig. 1). The findings by conventional microscopy, however, were indistinguishable from those of lupus erythematosus (Figs. 2A, 2B and 2C). The patient was asymptomatic and, except for slight increase in SGOT, his laboratory values and immunologic profile were normal or negative. The twin brother and the father of the patient had skin lesions similar to his. A biopsy specimen was taken from a lesion in the skin of the twin brother, the histopathologic features of which also were consonant with lupus erythematosus. The more we read about Degos' disease, the more enigmatic it became, thereby prompting us to review all that has been written about it. Having done that, we now set forth a view of the subject different from any account given before.