English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Plus monthly

Global: Zendy Tools annual

Global: Zendy Tools monthly

Global: Zendy Free Plan

Degos' disease, or malignant atrophic papulosis, is a rare and often fatal multisystem vasculopathy of unknown etiology. The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim. Involvement of the gastrointestinal tract is observed in 50% of cases, with intestinal perforation being the most common cause of death. Other organ systems can also be affected; 20% of cases involve the central nervous system. Systemic manifestations usually develop from weeks to years after onset of skin lesions or, in rare instances, may precede skin lesions. In the patient with Degos' disease reported in this article, the characteristic skin lesions developed during pregnancy, a precipitating event not previously reported. She has survived an unusually long time (10 years) without visceral or neurological involvement, despite florid cutaneous lesions. Moreover, we could detect the presence of antiphospholipid antibodies, the significance of which are currently unclear. These observations therefore confirm that there may be a strictly cutaneous form of Degos' disease with a favourable prognosis.

Benign Degos' Disease Developing During Pregnancy and Followed for 10 Years