Rare Diseases Symptoms Automatic Extraction

[Degos' disease as a cutaneous manifestation of systemic lupus erythematosus].

[malignant atrophic papulosis]

Degos' disease or malignant atrophic papulosis is a rare vasculopathy characterized by the presence of a typical skin lesion and visceral vascular involvement of small vessels, mainly of the digestive tract or central nervous system. The most interesting fact in this disease is the benign appearance of cutaneous lesion, hiding the occlusion of skin and visceral vessels. The author reports the case of a female patient with systemic lupus erythematosus for eight years. During her follow up, generalized skin papules were observed on the trunk and limbs, sparing her face, hands and feet, compatible with Degos' disease. Additional imaging investigation excluded systemic involvement of the disease. Treatment with acetylsalicylic acid prevented the appearance of new cutaneous manifestations and the patient remains clinically stable on the Outpatient Clinic without complications, until this moment. Malign atrophic papulosis is a rare disease with a poor prognosis. However, its association with systemic lupus erythematosus seems to follow a more benign course, without the typical visceral involvement.

Diseases presenting "systemic lupus erythematosus" symptom

  • acute rheumatic fever
  • familial mediterranean fever
  • focal myositis
  • hodgkin lymphoma, classical
  • inclusion body myositis
  • malignant atrophic papulosis
  • oligodontia
  • papillon-lefèvre syndrome
  • primary hyperoxaluria type 1
  • pyomyositis
  • sneddon syndrome
  • werner syndrome
  • x-linked adrenoleukodystrophy

This symptom has already been validated