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 "poor prognosis" symptom

  • adrenal incidentaloma
  • alexander disease
  • alpha-thalassemia
  • carcinoma of the gallbladder
  • cholangiocarcinoma
  • cushing syndrome
  • cutaneous mastocytosis
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • esophageal squamous cell carcinoma
  • hodgkin lymphoma, classical
  • junctional epidermolysis bullosa
  • kindler syndrome
  • liposarcoma
  • locked-in syndrome
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • megacystis-microcolon-intestinal hypoperistalsis syndrome
  • papillon-lefèvre syndrome
  • pleomorphic liposarcoma
  • primary effusion lymphoma
  • pyomyositis
  • sneddon syndrome
  • systemic capillary leak syndrome
  • von hippel-lindau disease
  • waldenström macroglobulinemia
  • well-differentiated liposarcoma
  • werner syndrome
  • wiskott-aldrich syndrome
  • zellweger syndrome

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