Bogenrieder, Thomas and Kuske, Marion and Landthaler, Michael and Stolz, Wilhelm (2002) Benign Degos' disease developing during pregnancy and followed for 10 years. ACTA DERMATO-VENEREOLOGICA, 82 (4). pp. 284-287. ISSN 0001-5555
Full text not available from this repository. (Request a copy)Abstract
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 anti-phospholipid 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | MALIGNANT ATROPHIC PAPULOSIS; PATIENT; VASCULITIS; antiphospholipid antibodies; Degos' disease; malignant atrophic papulosis; porcelain-white scars; pregnancy |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Dermatologie und Venerologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 12 Oct 2021 13:00 |
| Last Modified: | 12 Oct 2021 13:00 |
| URI: | https://pred.uni-regensburg.de/id/eprint/40090 |
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