Degos disease, not just a scar: lethal outcome inspite of immunomodulatory therapy

Abstract

Degos disease (DD) or papulosis atrophicans maligna, is an extremely infrequent thrombotic microangiopathy of unknown etiology⁽¹⁾. Clinically, it is characterized by small erythematous papules with an atrophic center that may simulate a simple scar and distinct histopathological feature that is not always present. Due to the rarity of this entity, diagnosis may go unnoticed with the first onset of cutaneous lesions. Therefore, high clinical suspicion and adequate clinicopathological correlation are required to establish an early diagnosis and consequently treatment.

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