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Malignant Papulosis with Atrophy (Degos): Fatal Cutaneointestinal Syndrome

Malignant Papulosis with Atrophy (Degos): Fatal Cutaneointestinal Syndrome Abstract Enough cases of the fatal cutaneointestinal syndrome malignant papulosis with atrophy have now been reported so that it can be considered a clinical and pathologic entity. When, in 1942, Degos et al.1 presented their first patient, then having only skin lesions, they were unaware of the future fatal outcome, but nevertheless felt that the patient had a previously undescribed disease of the skin. Several months later the man died after a severe acute gastrointestinal condition. At operation, and later at autopsy, there was no perforation of the intestine, but many yellow lesions of the small intestine were found. These atrophic lesions of the intestine were identifiable, both microscopically and macroscopically with the atrophic skin lesions, and were assumed to have the same causation. The history of development, the character and type of the eruptive lesions are very uniform in all the cases reported. Degos' first case illustrates the course References 1. Degos, R.; Delort, J., and Tricot, R.: Dermatite papulo-squameuse atrophiante , Bull. Soc. franç. dermat. et syph. 49:148-150 and 281, 1942. 2. Degos, R.: Papulose atrophiante maligne: (Syndrome cutanéo-intestinal mortel) , Ann. dermat. et syph. 79:410-417 ( (July) -Aug.) 1952. 3. Degos, R.: A propos de la papulose atrophiante maligne: (Syndrome cutanéo-intestinal mortel) , Bull. Soc. franç. dermat. et syph . 61:211-213 ( (May) -June) 1954. 4. Kölhmeier, W.: Multiple Hautnekrosen bei Thrombangiitis obliterans , Arch. f. Dermat. u. Syph. 181:783-792, 1941.Crossref 5. Lausecker, H.: Beitrag zur intestinalen Form der Thrombangiitis obliterans mit Hauterscheinungen , Acta dermato-venereol. 29:368-386, 1949. 6. Tzanck, Civatte et Sidi: Ulérythème porcelaine en gouttes , Bull. Soc. franç. dermat. et syph. 55:10-12 ( (Jan.) -Feb.) 1948. 7. Basset, A.; Guilaine, R., et Montfort, J.: La papulose atrophiante maligne de Degos: Étude anatomo-clinique , Presse méd. 64:2193-2196 ( (Dec.) 25) 1956. 8. Cottini, G. B., and Randazzo, S. D.: Sindrome cutaneo-mucoso-intestinale. (Contributo allo studio della papulosi atrofizzante maligna di Degos.) Minerva dermat. 31:1-9, 1956. 9. Stauffer, H., and Miescher, G.: Papulose atrophiante maligne (Degos) (Thrombangitis cutaneo-intestinalis disseminata) , Hautarzt 8:4-7 ( (Jan.) ) 1957. 10. Midana, A., and Zina, G.: Papulosis atrofizzante maligna di Degos a decorso benigno , Dermatologica 115:681-684, 1957.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Dermatology American Medical Association

Malignant Papulosis with Atrophy (Degos): Fatal Cutaneointestinal Syndrome

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Publisher
American Medical Association
Copyright
Copyright © 1960 American Medical Association. All Rights Reserved.
ISSN
0096-5359
DOI
10.1001/archderm.1960.03730020017003
Publisher site
See Article on Publisher Site

Abstract

Abstract Enough cases of the fatal cutaneointestinal syndrome malignant papulosis with atrophy have now been reported so that it can be considered a clinical and pathologic entity. When, in 1942, Degos et al.1 presented their first patient, then having only skin lesions, they were unaware of the future fatal outcome, but nevertheless felt that the patient had a previously undescribed disease of the skin. Several months later the man died after a severe acute gastrointestinal condition. At operation, and later at autopsy, there was no perforation of the intestine, but many yellow lesions of the small intestine were found. These atrophic lesions of the intestine were identifiable, both microscopically and macroscopically with the atrophic skin lesions, and were assumed to have the same causation. The history of development, the character and type of the eruptive lesions are very uniform in all the cases reported. Degos' first case illustrates the course References 1. Degos, R.; Delort, J., and Tricot, R.: Dermatite papulo-squameuse atrophiante , Bull. Soc. franç. dermat. et syph. 49:148-150 and 281, 1942. 2. Degos, R.: Papulose atrophiante maligne: (Syndrome cutanéo-intestinal mortel) , Ann. dermat. et syph. 79:410-417 ( (July) -Aug.) 1952. 3. Degos, R.: A propos de la papulose atrophiante maligne: (Syndrome cutanéo-intestinal mortel) , Bull. Soc. franç. dermat. et syph . 61:211-213 ( (May) -June) 1954. 4. Kölhmeier, W.: Multiple Hautnekrosen bei Thrombangiitis obliterans , Arch. f. Dermat. u. Syph. 181:783-792, 1941.Crossref 5. Lausecker, H.: Beitrag zur intestinalen Form der Thrombangiitis obliterans mit Hauterscheinungen , Acta dermato-venereol. 29:368-386, 1949. 6. Tzanck, Civatte et Sidi: Ulérythème porcelaine en gouttes , Bull. Soc. franç. dermat. et syph. 55:10-12 ( (Jan.) -Feb.) 1948. 7. Basset, A.; Guilaine, R., et Montfort, J.: La papulose atrophiante maligne de Degos: Étude anatomo-clinique , Presse méd. 64:2193-2196 ( (Dec.) 25) 1956. 8. Cottini, G. B., and Randazzo, S. D.: Sindrome cutaneo-mucoso-intestinale. (Contributo allo studio della papulosi atrofizzante maligna di Degos.) Minerva dermat. 31:1-9, 1956. 9. Stauffer, H., and Miescher, G.: Papulose atrophiante maligne (Degos) (Thrombangitis cutaneo-intestinalis disseminata) , Hautarzt 8:4-7 ( (Jan.) ) 1957. 10. Midana, A., and Zina, G.: Papulosis atrofizzante maligna di Degos a decorso benigno , Dermatologica 115:681-684, 1957.Crossref

Journal

A.M.A. Archives of DermatologyAmerican Medical Association

Published: Feb 1, 1960

References