Acta Scientific Medical Sciences (ISSN: 2582-0931)

Case Report Volume 4 Issue 1

Dego`S Disease with Pleural Effusion. Case Report and Review of Literature

Fadil Gradica1*, Daniela Xhemalaj1, Lutfi Lisha1, Dhimitraq Argjiri1, Alma Cani1, Fahri Kokici1 Alma Teferici1, Flora Gradica2, Perlat Kapisyzi1 and Arben Gjata3

1University Hospital "Shefqet Ndroqi", Tirana-Albania
2Public Pharmacy, Tirana-Albania
3Visceral Surgery University Center “Mother Theresa" Tirana-Albania

*Corresponding Author: Fadil Gradica, University Hospital "Shefqet Ndroqi", Tirana-Albania.

Received: November 19, 2019; Published: December 06, 2019

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Abstract

  Degos’ disease, also known as “malignant atrophic papulosis” is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology wich was first described by Dego in 1942 [1], but another case, reported in 1941 by Köhlmeier, who interpreted it as thromboangiitis obliterans of the mesenteric vessels [2] This is an arteriopathy which involves vessels of small-caliber. Dego disease mostly affects the skin. It occurs as a limited benign, cutaneous form and in a systemic variant potentially lethal multiorgan. Male to female ratio of this disease is (3:1), and a few cases of familial involvement have been reported [3-7]. In 60% of reported cases there has been an involvement of the gastrointestinal tract and other organs [8]. Up to now, fewer than 50 living patients are known worldwide, and fewer than 200 cases reported in medical literature, because it is underdiagnosed due to the rarity of this nosology [9,10]. Patients shows symptoms between of 20- 50 years; however, symtoms have been reported even as 8 months [1,6].

Keywords: Malignant Atrophic Papulosis (MAP); Pleural Effusion; Dego

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References

  1. Degos R., et al. “Dermatitepapular atrophicans. Dermatitepapulo-squameuseatrophiante”. Bulletin De La Societe Francaise De Dermatologie Et De Syphiligraphie 49 (1942): 148-281.
  2. Köhlmeier W. “Multiple skin necrosis in thromboangiitis obliterans. Multiple HautnekrosenbeiT hromboangiitis obliterans”. Arch Dermatol Syphilol 181 (1941): 792-793.
  3. Degos R. “Malignant atrophic papulosis”. British Journal of Dermatology 100 (1979): 21-35.
  4. González JA., et al. “Papulosis atrofiantemaligna de Degos (observacion de um casoafectandoa um lactante). [Degos’ malignant atrophic papulosis (observation of a case in an infant)”. Actas Dermo-Sifiliográficas 5-6 (1975): 317-319.
  5. Habbema L., et al. “Familial malignant atrophic papulosis (Degos’ disease) - additional evidence for heredity and a benign course”. British Journal of Dermatology 114 (1986): 134-135.
  6. Kisch LS and Bruynzeel DP. “Six cases of malignant atrophic papulosis (Degos’ disease) occurring in one family”. British Journal of Dermatology 111 (1984): 469-471.
  7. Newton JA and Black MM. “Familial malignant atrophic papulosis”. Clinical and Experimental Dermatology 9 (1984): 298-299.
  8. Vicktor C and Schultz-Ehrenburg U. “Papulosis maligna atrophicans (Köhlmeier-Degos): Diagnose, Therapie, Verlauf. [Malignant atrophic papulosis (Köhlmeier-Degos): diagnosis, therapy and course]”. Hautarzt 52 (2001): 734-737.
  9. Schwaiger T., et al. “Autoimmune pancreatitis in MRL/Mp mice is a T-cell-mediated disease responsive to cyclosporine A and rapamycin treatment”. Gut 3 (2014): 494-505.
  10. Moulin G., et al. “Benign forms of Degos’ malignant atrophic papulosis]”. Ann Dermatol Venereol 115 (1988): 1289-1290.
  11. Burg G., et al. “Malignant atrophic papulosis”. Hautarzt 40 (1989): 480-485.
  12. Magrinat G., et al. “The clinical manifestations of Degos’ syndrome”. Archives of Pathology and Laboratory Medicine 113 (1989): 354-362.
  13. Winkelmann RK., et al. “Malignant papulosis of skin and cerebrum”. Archives of Dermatological 87 (1963): 54-62.
  14. Subbiah P., et al. “Skin lesion with a fatal neurologic outcome (Degos' disease)”. Neurology 46 (1996): 636-640.
  15. Rosemberg S., et al. “Childhood Degos’ disease with prominent neurological symptoms: report of a clinicopathological case”. Journal of Child Neurology 3 (1988): 42-46.
  16. Chest1 (1978):79-84.
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Citation

Citation: Fadil Gradica., et al. "Dego`S Disease with Pleural Effusion. Case Report and Review of Literature".Acta Scientific Medical Sciences 4.1 (2020): 27-30.



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