Is Degos' disease a clinical and histological end point rather than a specific disease?

Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near "pathognomonic" appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied. We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions. Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.