Rare Diseases Symptoms Automatic Extraction

Degos' disease with delayed involvement of the gastrointestinal tract.

[malignant atrophic papulosis]

A 75-year-old woman presented with pink papules over the trunk and limbs. Over the next few weeks, the papules became umbilicated and then developed into porcelain-white lesions with a rose or violaceous rim. A biopsy of one of these lesions revealed superficial and deep dermal lymphohistiocytic inflammation, mainly perivascular with a central zone of infarction consistent with Degos' disease. Three years later, she presented with increasing abdominal pain, anaemia, raised inflammatory markers and an abdominal bruit. Angiography showed splanchnic vessel stenosis possibly secondary to vasculitis and she underwent successful balloon dilatation. Treatment with prednisolone was commenced. There has been no recurrence of lesions in the 12 months since the balloon dilatation.

Diseases presenting "vasculitis" symptom

  • coats disease
  • dracunculiasis
  • dystrophic epidermolysis bullosa
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • familial mediterranean fever
  • focal myositis
  • inclusion body myositis
  • malignant atrophic papulosis
  • papillon-lefèvre syndrome
  • pyruvate dehydrogenase deficiency
  • scrub typhus
  • sneddon syndrome
  • typhoid
  • waldenström macroglobulinemia

This symptom has already been validated