Abstract
Degos disease is a rare idiopathic vasculopathy with multisystem involvement. We present a previously healthy 40-year-old woman who developed spontaneous small bowel infarctions and perforations 17 months after presentation with characteristic skin lesions. The abdominal CT findings of Degos disease, which have not been previously depicted in the radiology literature, are discussed.
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Kohlmeier W (1941) Multiple Hauntnekrosen bei thromboangiitis obiterans. Arch Dermatol Syphilol 181:783–792
Degos R, Delort J, Tricot R (1942) Dermatite papulosquameuse atrophiante. Bull Soc Fr Dermatol Syphiligr 49:148–150
Feuerman EJ, Dollberg L, Salvador O (1970) Malignant atrophic papulosis with mucin in the dermis. A clinical and pathological study, including autopsy. Arch Pathol 90:310–315
Nomland R, Layton JM (1960) Malignant papulosis with atrophy (Degos); fatal cutaneointestinal syndrome. Arch Dermatol 81:181–188
Dastur DK, Singhal BS, Shroff HJ (1981) CNS involvement in malignant atrophic papulosis (Kohlmeier–Degos disease): vasculopathy and coagulopathy. J Neurol Neurosurg Psychiatry 44:156–160
Amato C, Ferri R, Elia M, et al. (2005) Nervous system involvement in Degos disease. AJNR Am J Neuroradiol 26:646–649
Fernandez-Perez ER, Grabscheid E, Scheinfeld NS (2005) A case of systemic malignant atrophic papulosis (Kohlmeier–Degos disease). J Natl Med Assoc 97:421–425
Yukiiri K, Mizushige K, Ueda T, et al. (2000) Degos disease with constrictive pericarditis: a case report. Jpn Circ J 64:464–467
Magrinat G, Kerwin KS, Gabriel DA (1989) The clinical manifestations of Degos syndrome. Arch Pathol Lab Med 113:354–362
Casparie MK, Meyer JW, van Huystee BE, et al. (1991) Endoscopic and histopathologic features of Degos disease. Endoscopy 23:231–233
Bilbao JI, Garcia Delgado F, Idoate M, et al. (1986) Degos syndrome. Intestinal involvement as demonstrated by digital angiography: a case. J Radiol 67:711–713
Strole WE Jr, Clark WH Jr, Isselbacher KJ (1967) Progressive arterial occlusive disease (Kohlmeier-Degos). A frequently fatal cutaneosystemic disorder. N Engl J Med 276:195–201
Scheinfeld N (2005) Degos disease. http://www.eMedicine.com/derm/topic931.htm February 3
Burg G, Vieluf D, Stolz W, et al. (1989) Malignant atrophic papulosis (Kohlmeier–Degos disease). Hautarzt 40:480–485
Ball E, Newburger A, Ackerman AB (2003) Degos disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol 25:308–320
Scheinfeld N (2005) Degos disease is probably a distinct entity: a review of clinical and laboratory evidence. J Am Acad Dermatol 52:375–376; author reply 376–378
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Amaravadi, R.R., Tran, T.M., Altman, R. et al. Small bowel infarcts in Degos disease. Abdom Imaging 33, 196–199 (2008). https://doi.org/10.1007/s00261-007-9225-1
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DOI: https://doi.org/10.1007/s00261-007-9225-1