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Journal of Medical Case Reports

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Open Access 01-12-2020 | Case report

Degos disease: a case report and review of the literature

Authors: Santosh Tummidi, Prabhakaran Nagendran, Swaroopa Gedela, Jami Rupa Ramani, Arundhathi Shankaralingappa

Published in: Journal of Medical Case Reports | Issue 1/2020

Abstract

Background

Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved.

Case presentation

A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of depigmented painful lesions. A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease.

Conclusion

The diagnosis of Degos disease is usually based on the presence of the pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of necrosis with thrombotic occlusion of the small arterioles. No specific treatment is currently available for this disease.

Yaghoobi Notash Ali YN, Hamed M, Azita N. Lethal systemic Degos disease with prominent cardiopulmonary Involvement. Indian J Dermatol. 2011;56(5):564–7.CrossRefPubMedPubMedCentral

Kohlmeier W. Multiple Hautnekrosen bei Thrombangiitis obliterans. Arch Dermatol. 1941;181:783–92.CrossRef

Degos R, Delort J, Tricot R. Dermatite papulo-squameuseatrophiante. Bull Soc Fr Dermatol Syphiligr. 1942;49:148–50.

Stavorn T, Chanprapaph K. Degos-like lesions in association with connective tissue diseases: a report of three cases and literature review. Clin Cosmet Investig Dermatol. 2019;12:815–22.CrossRefPubMedPubMedCentral

Sharma S, Brennan B, Naden R, Whelan P. A case of Degos disease in pregnancy. Obstet Med. 2016;9(4):167–8.CrossRefPubMedPubMedCentral

Padhiyar JK, Patel NH, Gajjar TP, Buch MD. Dego’s disease in a female child with anti-Scl-70 antibody positivity. Indian J Paediatr Dermatol. 2018;19:80–2.CrossRef

Ball E, Newburger A, Ackerman AB. Dego’s disease: a distinctive pattern of disease, chiefly of lupus erythematosus and not a specific disease per se. Am J Dermatopathol. 2003;25:308–20.CrossRefPubMed

Pirolla E, Fregni F, Miura IK, Misiara AC, Almeida F, Zanoni E. Degos disease – malignant atrophic papulosis or cutaneo-intestinal lethal syndrome: rarity of the disease. Clin Exp Gastroenterol. 2015;8:141–7.CrossRefPubMedPubMedCentral

Rice AS, Zedek D. Malignant atrophic papulosis (Degos disease). In: StatPearls. Treasure Island: StatPearls Publishing; 2020.

10.

Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC. Malignant and benign forms of atrophic papulosis (Kohlmeier-Degos disease): systemic involvement determines the prognosis. Br J Dermatol. 2014;170(1):110–5.CrossRefPubMed

11.

Li Z, Jin P, Wang B, Feng S. Two cases of Degos disease with different prognosis. Postepy Dermatol Alergol. 2014;31(6):425–7.CrossRefPubMedPubMedCentral

12.

Mareschal A, Razanamahery J, Aubin F. Malignant atrophic papulosis. JAMA Dermatol. 2020;156(2):204.CrossRefPubMed

Title: Degos disease: a case report and review of the literature
Authors: Santosh Tummidi
Prabhakaran Nagendran
Swaroopa Gedela
Jami Rupa Ramani
Arundhathi Shankaralingappa
Publication date: 01-12-2020
Publisher: BioMed Central
Published in: Journal of Medical Case Reports / Issue 1/2020
Electronic ISSN: 1752-1947
DOI: https://doi.org/10.1186/s13256-020-02514-6

At a glance: The STEP trials

Springer Medicine

Degos disease: a case report and review of the literature

Abstract

Background

Case presentation

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Case presentation

Conclusion

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