All issues > Volume 34(12); 1991

Original Article

J Korean Pediatr Soc. 1991;34(12):1724-1729. Published online December 31, 1991.

A case of malignant atrophic papulosis (Degos' disease).

Sung Ku Lee¹, In Seok Lim¹, Chul Ha Kim¹, Dong Keun Lee¹, Eung Sang Choi¹, Byoung Hoon Yoo¹, Gae Yong Song²

¹Department of Pediatrics, College of Medicine, Chung Ang University, Seoul, Korea
²Department of Pathology, College of Medicine, Chung Ang University, Seoul, Korea

Received: April 1, 1991;  Accepted: July 10, 1991.

Abstract

Malignant atrophic papulosis (Degos’ disease) is characterized by porcelain-white skin lesions. It has unknown etiology and bad prognosis. Death usually occurs from multiple intestinal perforations weeks to years after onset. We experienced a case of Degos’ disease in a 12-year-old male patient who had porcelain white papules on face, trunk and extremities. Histopathologic studies showed broad epidermal necrobiosis with thrombotic arteriolar occlusion. A brief review of related literature is also presented.

Keywords :Malignant atrophic papulosis