Systemic Dego`s Disease. Our Approach to the Diagnosis and the Follow-Up. A Case reports.
AJTES Vol 4, No 2, July 2020
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Keywords

Degos’ disease,
malignant atrophic papulosis,
pleural effusion

How to Cite

GRADICA, F., & XHEMALAJ, D. (2020). Systemic Dego`s Disease. Our Approach to the Diagnosis and the Follow-Up. A Case reports. Albanian Journal of Trauma and Emergency Surgery, 4(2), 729-731. https://doi.org/10.32391/ajtes.v4i2.95

Abstract

Degos’ disease, also known as “malignant atrophic papulosis” is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology which 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).

It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. Degos disease occurs both in a limited benign, cutaneous form and in a potentially lethal multiorgan, systemic variant.

The disease has a male predominance (3:1), and sporadic cases of familial involvement have been recorded. (3-7). The involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of reported cases (8).

There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease (9,10).

 Most individuals develop symptoms between the ages of 20-50; however, cases outside of this age range have been reported as well, even as early as 8 months (1,6).

https://doi.org/10.32391/ajtes.v4i2.95
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References

Degos R, Delort J, Tricot R. [Dermatite papular atrophicans]. Dermatite papulo-squameuse atrophiante. Bull Soc Fr Dermatol Syphiligr. 1942; 49: 148–281.

Köhlmeier W. [Multiple skin necrosis in thromboangiitis obliterans]. Multiple Hautnekrosen bei Thromboangiitis obliterans. Arch Dermatol Syphilol. 1941; 181: 792–793.

Degos R. Malignant atrophic papulosis. Br J Dermatol. 1979; 100: 21–35.

González JA, Noguer S, Cabré J. Papulosis atrofiante maligna de Degos (observacion de um caso afectando a um lactante). [Degos’ malignant atrophic papulosis (observation of a case in an infant)] Actas Drmosifiliogr. 1975;66(5–6):317–319.

Habbema L, Kisch LS, Starink TM. Familial malignant atrophic papulosis (Degos’ disease) – additional evidence for heredity and a benign course. Br J Dermatol. 1986; 114: 134–135.

Kisch LS, Bruynzeel DP. Six cases of malignant atrophic papulosis (Degos’ disease) occurring in one family. Br J Dermatol. 1984; 111: 469–471.

Newton JA, Black MM. Familial malignant atrophic papulosis. Clin Exp Dermatol. 1984; 9: 298–299.

Vicktor C, Schultz-Ehrenburg U. Papulosis maligna atrophicans (Köhlmeier–Degos): Diagnose, Therapie, Verlauf. [Malignant atrophic papulosis (Köhlmeier-Degos): diagnosis, therapy and course] Hautarzt. 2001; 52: 734–737.

Schwaiger T, van den Brandt C, Fitzner B, et al. Autoimmune pancreatitis in MRL/Mp mice is a T-cell-mediated disease responsive to cyclosporine A and rapamycin treatment. Gut. 2014;63(3):494–505.

Moulin G. Les formes benignes de la papulose atrophiante maligna de Degos. [Benign forms of Degos’ malignant atrophic papulosis] Ann Dermatol Venereol. 1988; 115: 1289–1290.

Burg G, Vieluf D, Stolz W, et al. Malignant atrophic papulosis. Hautarzt. 1989; 40: 480–485.

Magrinat G, Kerwin KS, Gabriel DA. The clinical manifestations of Degos’ syndrome. Arch Pathol Lab Med. 1989; 113: 354–362.

Winkelmann RK, Howard FM, Perry HO, et al. Malignant papulosis of skin and cerebrum. Arch Dermatol. 1963; 87: 54–62.

Subbiah P, Wijdicks E, Muenter M. et al Skin lesion with a fatal neurologic outcome (Degos' disease). Neurology 199646636–640

Rosemberg S, Lopes MB, Sotto MN, Graudenz MS. Childhood Degos’ disease with prominent neurological symptoms: report of a clinicopathological case. J Child Neurol. 1988; 3: 42–46

Scheinfeld N. Commentary on ‘Degos disease: a C5b-9/interferon-α-mediated endotheliopathy syndrome’ by Magro et al: a reconsideration of Degos disease as hematologic or endothelial genetic disease. Dermatol Online J. 2011;17(8):6.

Meephansan J, Komine S, Hosoda S, et al. Possible involvement of SDF-1/CXCL 12 in the pathogenesis of Degos disease. J Am Acad Dermatol. 2013;68(1):138–143.

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