Abstract
BackgroundDegos 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 presentationA 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.ConclusionThe 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.
Highlights
Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause
Case presentation A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of painful depigmented lesions of 11⁄2 years’ duration
* Correspondence: born_vss@yahoo.co.in 1Department of Pathology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India Full list of author information is available at the end of the article history of joint pain for the last 7–8 years and a history of headache, giddiness, and ocular pain for the last 3 months
Summary
Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. Background Degos disease (DD), called “malignant atrophic papulosis” or “lethal cutaneous and gastrointestinal arteriolar thrombosis,” is a rare occlusive arteriopathy having unknown pathogenesis and involves predominantly smallcaliber vessels of the dermis, gastrointestinal tract, central nervous system, and occasionally other organs [1,2,3]. * Correspondence: born_vss@yahoo.co.in 1Department of Pathology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India Full list of author information is available at the end of the article history of joint pain for the last 7–8 years and a history of headache, giddiness, and ocular pain for the last 3 months.
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