Dego's disease in a female child with Anti-Scl-70 antibody positivity

Padhiyar, Jignaben Krunal; Patel, Nayan H; Gajjar, Trusha; Buch, Mansi D

doi:10.4103/ijpd.ijpd_84_17

Cited by 1 publication

(2 citation statements)

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“…The following year, Degos presented DD as a distinct entity and coined its name [ 3 ]. DD, also known as “malignant atrophic papulosis,” is a rare systemic vaso-occlusive disorder with unknown pathophysiology [ 6 ]. Some authors have stated that DD might result from coagulopathy, vasculitis, or endothelial cell dysfunction [ 5 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

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Degos disease: a case report and review of the literature

et al. 2020

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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.

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Section: Discussionmentioning

confidence: 99%

“…The diagnosis of malignant atrophic papulosis is usually based on the presence of pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of wedge necrosis with end arterial thrombotic occlusion of the small arteries and infraction of dermis [ 1 , 6 , 9 , 11 ].…”

Section: Discussionmentioning

confidence: 99%