title: [Primary systemic amyloidosis].
abstract: BACKGROUND
  Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another disease.
  
  
  METHODS
  We presented a 72-year-old male patient with periocular purpura ("racoon sign") and waxy papules, petechiae and ecchymoses on the neck and thoracic area. Purpuric macules were present also on the lips and tongue which was edematous (macroglossia). The skin lesions occurred two years earlier, the patient lost more than 15 kilograms of the body mass for less than a year. Immunoelectrophoresis of urine and serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. Histopathological examination of skin lesions showed Congo red positive deposits in the derm. Cardiac evaluation revealed the signs of heart failure, and renal evaluation revealed nephrotic syndrome, with excessive protein lost. He was treated with oral melphalan and prednisolone, and died 7 days after starting the therapy due to heart failure.
  
  
  CONCLUSIONS
  This patient considered as a rare case with systemic amyloidosis highlights the importance of histopathological and physical examination in any cases with periocular purpura, petechiae, ecchymoses and macroglossia.
authors: Klinicki centar Srbije, Institut za dermatovenerologiju, Beograd. drtanas@mail.com
authors: Srdan
authors: S
authors: Tanasilović
authors: Dubravka
authors: D
authors: Zivanović
authors: Milos
authors: M
authors: Nikolić
authors: Maja
authors: M
authors: Tomović
authors: Ivo
authors: I
authors: Elezović
authors: Ljiljana
authors: L
authors: Medenica
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male