Malignant Atrophic Papulosis Differential Diagnoses
- Author: L Campbell Levy, MD; Chief Editor: Julian Katz, MD more...
Kohlmeier W. Multiple Hautnekrosen bei thromboangitis obliterans. Arch Dermatol Syphilol. 1941;181:783-792.
Degos R, Delorot J, Tricot R. Dermatite paplulosquameuse atrophinate. Bull Soc Fr Dermatol Syphiligr. 1942;49:148-150.
Moss C, Wassmer E, Debelle G, et al. Degos disease: a new simulator of non-accidental injury. Dev Med Child Neurol. Aug 2009;51(8):647-50. [Medline].
Aizawa H, Takase Y, Inoue K, Murayama S, Mannen T. [An autopsy case of Degos disease with neurological symptoms--neuropathological observations and increased platelet aggregation] [Japanese]. Rinsho Shinkeigaku. Jan 1992;32(1):23-9. [Medline].
Amato C, Ferri R, Elia M, et al. Nervous system involvement in Degos disease. AJNR Am J Neuroradiol. Mar 2005;26(3):646-9. [Medline]. [Full Text].
Assier H, Chosidow O, Piette JC. Absence of antiphospholipid and anti-endothelial cell antibodies in malignant atrophic papulosis: a study of 15 cases. J Am Acad Dermatol. Nov 1995;33(5 Pt 1):831-3. [Medline].
Atchabahian A, Laisne MJ, Riche F, et al. Small bowel fistulae in Degos' disease: a case report and literature review. Am J Gastroenterol. Oct 1996;91(10):2208-11. [Medline].
Aydogan K, Alkan G, Karadogan et al. Painful penile ulceration in a patient with malignant atrophic papulosis. J Eur Acad Dermatol Venereol. Sep 2005;19(5):612-6. [Medline].
Ball E, Newburger A, Ackerman AB, et al. Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol. Aug 2003;25(4):308-20. [Medline].
Barlow RJ, Heyl T, Simson IW, Schulz EJ. Malignant atrophic papulosis (Degos' disease)--diffuse involvement of brain and bowel in an African patient. Br J Dermatol. Jan 1988;118(1):117-23. [Medline].
Beales IL. Malignant atrophic papulosis presenting as gastroparesis. Am J Gastroenterol. Dec 2001;96(12):3462. [Medline].
Bhagwat PV, Tophakhane RS, Shashikumar BM, Noronha TM, Naidu V. Three cases of Dowling Degos disease in two families. Indian J Dermatol Venereol Leprol. Jul-Aug 2009;75(4):398-400. [Medline].
Black MM. Malignant atrophic papulosis (Degos' disease). Int J Dermatol. Jul-Aug 1976;15(6):405-11. [Medline].
Black MM, Jones EW. Malignant atrophic papulosis (Degos' syndrome). Br J Dermatol. Sep 1971;85(3):290-2. [Medline].
Black MM, Nishioka K, Levene GM. The role of dermal blood vessels in the pathogenesis of malignant atrophic papulosis (Degos' disease). A study of two cases using enzyme histochemical, fibrinolytic, electron-microscopical and immunological techniques. Br J Dermatol. Mar 1973;88(3):213-9. [Medline].
Bogenrieder T, Kuske M, Landthaler M, Stolz W. Benign Degos' disease developing during pregnancy and followed for 10 years. Acta Derm Venereol. 2002;82(4):284-7. [Medline].
Burrow JN, Blumbergs PC, Iyer PV. Kohlmeier-Degos disease: a multisystem vasculopathy with progressive cerebral infarction. Aust N Z J Med. Feb 1991;21(1):49-51. [Medline].
Casparie MK, Meyer JW, van Huystee BE, Kneppelhout J, Mulder CJ. Endoscopic and histopathologic features of Degos' disease. Endoscopy. Jul 1991;23(4):231-3. [Medline].
Chave TA, Varma S, Patel GK, Knight AG. Malignant atrophic papulosis (Degos' disease): clinicopathological correlations. J Eur Acad Dermatol Venereol. Jan 2001;15(1):43-5. [Medline].
Chung HY, Trendell-Smith NJ, Yeung CK, Mok MY. Degos' disease: a rare condition simulating rheumatic diseases. Clin Rheumatol. Jul 2009;28(7):861-3. [Medline].
Coskun B, Saral Y, Cicek D, Ozercan R. Benign cutaneous Degos' disease: a case report and review of the literature. J Dermatol. Aug 2004;31(8):666-70. [Medline].
Crowson AN, Magro CM, Usmani A, McNutt NS. Immunoglobulin A-associated lymphocytic vasculopathy: a clinicopathologic study of eight patients. J Cutan Pathol. Nov 2002;29(10):596-601. [Medline].
De Breucker S, Vandergheynst F, Decaux G. Inefficacy of intravenous immunoglobulins and infliximab in Degos' disease. Acta Clin Belg. Mar-Apr 2008;63(2):99-102. [Medline].
Degos R. Malignant atrophic papulosis. Br J Dermatol. Jan 1979;100(1):21-35. [Medline].
Delaney TJ, Black MM. Effect of fibrinolytic treatment in malignant atrophic papulosis. Br Med J. Aug 16 1975;3(5980):415. [Medline].
Drucker CR. Malignant atrophic papulosis: response to antiplatelet therapy. Dermatologica. 1990;180(2):90-2. [Medline].
Dubin HV, Stawiski MA. Systemic lupus erythematosus resembling malignant atrophic papulosis. Arch Intern Med. Aug 1974;134(2):321-3. [Medline].
Durie BG, Stroud JD, Kahn JA. Progressive systemic sclerosis with malignant atrophic papulosis. Arch Dermatol. Nov 1969;100(5):575-81. [Medline].
Egan R, Lessell S. Posterior subcapsular cataract in Degos disease. Am J Ophthalmol. Jun 2000;129(6):806-7. [Medline].
Englert HJ, Hawkes CH, Boey ML, et al. Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant. Br Med J (Clin Res Ed). Sep 8 1984;289(6445):576. [Medline].
Evans GH, Ribeiro BF. Degos' disease. A rare cause of multiple intestinal perforation. J R Coll Surg Edinb. Dec 1987;32(6):371-2. [Medline].
Farrell AM, Moss J, Costello C, et al. Benign cutaneous Degos' disease. Br J Dermatol. Oct 1998;139(4):708-12. [Medline].
Fernandez-Perez ER, Grabscheid E, Scheinfeld NS. A case of systemic malignant atrophic papulosis (Kohlmeier-Degos' disease). J Natl Med Assoc. Mar 2005;97(3):421-5. [Medline]. [Full Text].
Fruhwirth J, Mischinger HJ, Werkgartner G, Beham A, Pfaffenthaller EC. Kohlmeier-Degos's disease with primary intestinal manifestation. Scand J Gastroenterol. Oct 1997;32(10):1066-70. [Medline].
Gilaberte Y, Coscojuela C, Lezaun A, Marigil MA. Degos disease associated with protein S deficiency. Br J Dermatol. Sep 2005;153(3):666-7. [Medline].
Gonzalez Valverde FM, Menarguez Pina F, Ruiz JA, et al. Presentation of Degos syndrome as acute small-bowel perforation. Arch Surg. Jan 2003;138(1):57-8. [Medline].
Grilli R, Soriano ML, Izquierdo MJ. Panniculitis mimicking lupus erythematosus profundus: a new histopathologic finding in malignant atrophic papulosis (Degos disease). Am J Dermatopathol. Aug 1999;21(4):365-8. [Medline].
Guhl G, Diaz-Ley B, Delgado Y, et al. Wegener's granulomatosis: a new entity in the growing differential diagnosis of Degos' disease. Clin Exp Dermatol. Jul 2009;34(5):e1-3. [Medline].
Guven FO, Bozdag KE, Ermete M, Karaman A. Degos' disease. Int J Dermatol. May 2000;39(5):361-2. [Medline].
Hall-Smith P. Malignant atrophic papulosis (Degos' disease). Two cases occurring in the same family. Br J Dermatol. Nov 1969;81(11):817-22. [Medline].
Harvell JD, Williford PL, White WL. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve. Am J Dermatopathol. Apr 2001;23(2):116-23. [Medline].
High WA, Aranda J, Patel SB, et al. Is Degos' disease a clinical and histological end point rather than a specific disease?. J Am Acad Dermatol. Jun 2004;50(6):895-9. [Medline].
Howsden SM, Hodge SJ, Herndon JH. Malignant atrophic papulosis of Degos. Report of a patient who failed to respond to fibrinolytic therapy. Arch Dermatol. Nov 1976;112(11):1582-8. [Medline].
Kanekura T, Uchino Y, Kanzaki T. A case of malignant atrophic papulosis successfully treated with nicotine patches. Br J Dermatol. Sep 2003;149(3):660-2. [Medline].
Katz SK, Mudd LJ, Roenigk HH Jr. Malignant atrophic papulosis (Degos' disease) involving three generations of a family. J Am Acad Dermatol. Sep 1997;37(3 pt 1):480-4. [Medline].
Kim DW, Kang SB, Lee KH, et al. Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case. Surg Today. 2008;38(9):866-70. [Medline].
Kirkup ME, Hunt SJ, Pawade J, Kennedy CT. Inflammatory linear vasculopathy mimicking Degos' disease. Br J Dermatol. Jun 2004;150(6):1225-7. [Medline].
Kisch LS, Bruynzeel DP. Six cases of malignant atrophic papulosis (Degos' disease) occurring in one family. Br J Dermatol. Oct 1984;111(4):469-71. [Medline].
Kocheril SV, Blaivas M, Appleton BE, McCune WJ, Ike RW. Degos' disease mimicking vasculitis. Arthritis Rheum. Jun 15 2004;51(3):498-500. [Medline].
Lankisch MR, Johst P, Scolapio JS, Fleming CR. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis). Am J Gastroenterol. Apr 1999;94(4):1098-9. [Medline].
Liu CM, Harris RM, Hansen CD. Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis. Cutis. Feb 2005;75(2):101-4. [Medline].
Loewe R, Palatin M, Petzelbauer P. Degos disease with an inconspicuous clinical course. J Eur Acad Dermatol Venereol. Jul 2005;19(4):477-80. [Medline].
McLoud TC, Munechika H, Shaw R, Putman CE. Calcific constrictive pericarditis in Degos' disease. South Med J. May 1978;71(5):609-11. [Medline].
Melnik B, Hariry H, Vakilzadeh F, Gropp C, Sitzer G. [Malignant atrophic papulosis (Köhlmeier-Degos disease). Failure to respond to interferon alpha-2a, pentoxifylline and aspirin] [German]. Hautarzt. Sep 2002;53(9):618-21. [Medline].
Mensing C, Mensing H. [Degos atrophic malignant papulosis. Not always malignant!] [German]. Hautarzt. Jan 2002;53(1):42-6. [Medline].
Negahban K, Chern K. Cataracts associated with systemic disorders and syndromes. Curr Opin Ophthalmol. Dec 2002;13(6):419-22. [Medline].
Newton JA, Black MM. Familial malignant atrophic papulosis. Clin Exp Dermatol. May 1984;9(3):298-9. [Medline].
Ojeda Cuchillero RM, Sanchez Regana M, Umbert Millet P. Benign cutaneous Degos' disease. Clin Exp Dermatol. Mar 2003;28(2):145-7. [Medline].
Paolaggi JA, Daniel F, Mugnier B, Debray C. [Digestive manifestations of malignant atrophying papulosis (Degos' disease). Value of laparoscopy] [French]. Ann Med Interne (Paris). Nov 1970;121(11):965-9. [Medline].
Pierce RN, Smith GJ. Intrathoracic manifestations of Degos' disease (malignant atrophic papulosis). Chest. Jan 1978;73(1):79-84. [Medline].
Pinault AL, Barbaud A, Weber-Muller F, Schmutz JL. [Benign familial Degos disease] [French]. Ann Dermatol Venereol. Nov 2004;131(11):989-93. [Medline].
Plantin P, Labouche F, Sassolas B, Delaire P, Guillet G. Degos' disease: a 10-year follow-up of a patient without visceral involvement. J Am Acad Dermatol. Jul 1989;21(1):136-7. [Medline].
Powell J, Bordea C, Wojnarowska F. Benign familial Degos disease worsening during immunosuppression. Br J Dermatol. Sep 1999;141(3):524-7. [Medline].
Premalatha S, Yesudian P, Janaki VR, Rao NR, Thambiah AS. Malignant atrophic papulosis (Degos' syndrome): first case report from India [letter]. Clin Exp Dermatol. Sep 1980;5(3):370-1. [Medline].
Rajagopalan B, Pulimood S, George S, Jacob M. Erythrokeratoderma en cocardes. Clin Exp Dermatol. May 1999;24(3):173-4. [Medline].
Requena L, Farina C, Barat A. Degos disease in a patient with acquired immunodeficiency syndrome. J Am Acad Dermatol. May 1998;38(5 Pt 2):852-6. [Medline].
Rivoire MJ. [Degos' malignant atrophic papulosis. Outcome after 28 months' treatment with anticoagulants] [French]. Bull Soc Fr Dermatol Syphiligr. 1971;78(1):18-9. [Medline].
Rodriguez MA. Ischemic colitis and malignant atrophic papulosis. Am J Gastroenterol. Feb 1977;67(2):163-6. [Medline].
Scheinfeld N. Degos' disease is probably a distinct entity: a review of clinical and laboratory evidence. J Am Acad Dermatol. Feb 2005;52(2):375-6; author reply 376-8. [Medline].
Scheinfeld N. Pairing and comparing nine diseases with Degos disease (malignant atrophic papulosis): an attempt to illustrate our understanding and direct future inquiry. Dermatol Online J. Jan 15 2009;15(1):10. [Medline].
Shimazu S, Imai H, Kokubu S, et al. [Long-term survival in malignant atrophic papulosis: a case report and review of the Japanese literature] [Japanese]. Nippon Geka Gakkai Zasshi. Oct 1988;89(10):1748-51. [Medline].
Sidi E, Reinberg A, Spinasse JB. Lethal cutaneous and gastrointestinal arteriolar thrombosis (malignant atrophying papulosis of Degos). JAMA. 1960;174:1170-3.
Snow JL, Muller SA. Degos syndrome: malignant atrophic papulosis. Semin Dermatol. Jun 1995;14(2):99-105. [Medline].
Soter NA, Murphy GF, Mihm MC Jr. Lymphocytes and necrosis of the cutaneous microvasculature in malignant atrophic papulosis: a refined light microscope study. J Am Acad Dermatol. Nov 1982;7(5):620-30. [Medline].
Stahl D, Thomsen K, Hou-Jensen K. Malignant atrophic papulosis: treatment with aspirin and dipyridamole. Arch Dermatol. Nov 1978;114(11):1687-9. [Medline].
Strole WE Jr, Clark WH Jr, Isselbacher KJ. Progressive arterial occlusive disease (Kohlmeier-Degos). A frequently fatal cutaneosystemic disorder. N Engl J Med. Jan 26 1967;276(4):195-201. [Medline].
Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos' disease). Neurology. Mar 1996;46(3):636-40. [Medline].
Sugai F, Sumi H, Hara Y, et al. [An autopsy case of Degos' disease with ascending thoracic myelopathy] [Japanese]. Rinsho Shinkeigaku. Dec 1998;38(12):1049-53. [Medline].
Thomas RK, Nithyanandam S, Rawoof BA, Rajendran SC. Malignant atrophic papulosis. Report of a case with multiple ophthalmic findings. Indian J Ophthalmol. Sep 2003;51(3):260-3. [Medline].
Thomson KF, Highet AS. Penile ulceration in fatal malignant atrophic papulosis (Degos' disease). Br J Dermatol. Dec 2000;143(6):1320-2. [Medline].
Torrelo A, Sevilla J, Mediero IG, et al. Malignant atrophic papulosis in an infant. Br J Dermatol. May 2002;146(5):916-8. [Medline].
Tribble K, Archer ME, Jorizzo JL. Malignant atrophic papulosis: absence of circulating immune complexes or vasculitis. J Am Acad Dermatol. Aug 1986;15(2 Pt 2):365-9. [Medline].
Vazquez-Doval FJ, Ruiz de Erenchun F, Paramo JA. Malignant atrophic papulosis. A report of two cases with altered fibrinolysis and platelet function. Clin Exp Dermatol. Sep 1993;18(5):441-4. [Medline].
Vicktor C, Schultz-Ehrenburg U. [Malignant atrophic papulosis (Kohlmeier-Degos): diagnosis, therapy and course] [German]. Hautarzt. Aug 2001;52(8):734-7. [Medline].
Wachter T, Rose C, Bröcker EB, Leverkus M. [Benign course of malignant atrophic papulosis (Kohlmeier-Degos disease): lack of vessel occlusion as good prognostic sign?] [German]. J Dtsch Dermatol Ges. May 2003;1(5):374-7. [Medline].
Ward SK, Roenigk HH, Gordon KB. Dermatologic manifestations of gastrointestinal disorders. Gastroenterol Clin North Am. Sep 1998;27(3):615-36, vi. [Medline].
Yag-Howard C, Shenefelt PD. Multiple pink papules with white, depressed centers. Malignant atrophic papulosis (Degos disease). Arch Dermatol. Feb 1998;134(2):233, 236. [Medline].
Yoshikawa H, Maruta T, Yokoji H, et al. Degos' disease: radiological and immunological aspects. Acta Neurol Scand. Nov 1996;94(5):353-6. [Medline].
Yukiiri K, Mizushige K, Ueda T, et al. Degos' disease with constrictive pericarditis: a case report. Jpn Circ J. Jun 2000;64(6):464-7. [Medline].
Zamiri M, Jarrett P, Snow J. Benign cutaneous Degos disease. Int J Dermatol. Aug 2005;44(8):654-6. [Medline].

