The cutaneous lesions of malignant atrophic papulosis (Degos disease) are said to be unique1 and so characteristic that the diagnosis can be made easily from a photograph.2
In 1942 Degos et al3 described this disease which was subsequently named "malignant atrophic papulosis," and it was first described in the United States by Naylor et al in 1960.4 This disease had actually been described in 1941 by Kohlmeier5 who considered it to be thromboangiitis obliterans of the intestine with skin lesions. There have been 32 cases of this disease reported in the world literature,6-9 and it is apparent that it is a multisystemic disease with cutaneous involvement.
The patient whose case is reported here had cutaneous lesions and central nervous system (CNS) signs and symptoms that suggested the diagnosis of malignant atrophic papulosis. The skin lesions had a gross and histologic appearance suggestive of this
Dubin HV, Stawiski MA. Systemic Lupus Erythematosus Resembling Malignant Atrophic Papulosis. Arch Intern Med. 1974;134(2):321-323. doi:10.1001/archinte.1974.00320200131019