Rare Diseases Symptoms Automatic Extraction

Degos disease with dermatomyositis-like phenomenon: a diagnostic dilemma and a therapeutic challenge.

[malignant atrophic papulosis]

Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity.We report a case of a 50-year-old male patient with classic skin lesions and rapidly progressive fatal clinical course involving multiple organs associated with dermatomyositis-like features. Therapeutic challenge in such a case of secondary Degos disease with multiorgan dysfunction is discussed.Secondary Degos disease with underlying multisystem dysfunction is a rapidly fatal disease. The patient must be thoroughly investigated to watch for systemic involvement and aggressively treated preferably with antiplatelet drugs and immunosuppresants so as to moderate the severity of the disease. Corticosteroids are best avoided to prevent gastrointestinal perforation.

Diseases presenting "skin lesions" symptom

  • child syndrome
  • cowden syndrome
  • cutaneous mastocytosis
  • cystinuria
  • dystrophic epidermolysis bullosa
  • epidermolysis bullosa simplex
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • familial mediterranean fever
  • focal myositis
  • gm1 gangliosidosis
  • heparin-induced thrombocytopenia
  • hirschsprung disease
  • junctional epidermolysis bullosa
  • lamellar ichthyosis
  • liposarcoma
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • oligodontia
  • omenn syndrome
  • papillon-lefèvre syndrome
  • primary effusion lymphoma
  • proteus syndrome
  • severe combined immunodeficiency
  • sneddon syndrome
  • waldenström macroglobulinemia
  • werner syndrome
  • wiskott-aldrich syndrome

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