Degos disease with an inconspicuous clinical course

Degos disease is a rare systemic disorder with involvement of the skin and visceral organs, leading to death in about 50% of cases within 1 or 2 years. In recent years, several cases with cutaneous lesions only have been recognized.

We report on a young male patient presenting with single inconspicuous papules with bluish/black centres on the trunk and the upper limbs that, upon healing turn white. These lesions recurred on different locations over the past 6 years, and were never more than two to three at one time.

Histopathological examinations revealed archetypal features for Degos disease. The patient had no other complaints, neither visceral organs nor the central nerve system were involved. Laboratory examinations were within normal range.

This case increases the number of reports on a benign course of Degos disease. It raises the question if the 'malignant' and the 'benign' course of the disease represent two distinct diseases or variants of a systemic vasculitis with unknown cause.