Benign Degos' disease developing during pregnancy and followed for 10 years.
Acta Derm Venereol
; 82(4): 284-7, 2002.
Article
en En
| MEDLINE
| ID: mdl-12361134
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 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.
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Bases de datos:
MEDLINE
Asunto principal:
Complicaciones del Embarazo
/
Enfermedades Cutáneas Papuloescamosas
Tipo de estudio:
Prognostic_studies
Límite:
Female
/
Humans
/
Pregnancy
Idioma:
En
Revista:
Acta Derm Venereol
Año:
2002
Tipo del documento:
Article
País de afiliación:
Alemania