Degos disease: a case report and review of the literature

Journal of Medical Case Reports

Jami Rupa Ramani¹  Prabhakaran Nagendran¹  Santosh Tummidi²  Arundhathi Shankaralingappa²  Swaroopa Gedela² 

[1] Department of Dermatology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India;Department of Pathology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India;

关键词: Degos disease;    Malignant atrophic papulosis;    Skin biopsy;    Vasculopathy;   

DOI  :  10.1186/s13256-020-02514-6

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY