Primary systemic amyloidosis
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. Case report. We presented a 72-year-old male patient w...
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| Format: | Article |
| Language: | English |
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Military Health Department, Ministry of Defance, Serbia
2007-01-01
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| Series: | Vojnosanitetski Pregled |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdf |
| _version_ | 1811256506395394048 |
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| author | Tanasilović Srđan Živanović Dubravka Nikolić Miloš Tomović Maja Elezović Ivo Medenica Ljiljana |
| author_facet | Tanasilović Srđan Živanović Dubravka Nikolić Miloš Tomović Maja Elezović Ivo Medenica Ljiljana |
| author_sort | Tanasilović Srđan |
| collection | DOAJ |
| description | 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. Case report. 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. Conclusion. 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. |
| first_indexed | 2024-04-12T17:41:11Z |
| format | Article |
| id | doaj.art-2b3941d726ee41618aaf4305cabc1dc9 |
| institution | Directory Open Access Journal |
| issn | 0042-8450 |
| language | English |
| last_indexed | 2024-04-12T17:41:11Z |
| publishDate | 2007-01-01 |
| publisher | Military Health Department, Ministry of Defance, Serbia |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj.art-2b3941d726ee41618aaf4305cabc1dc92022-12-22T03:22:47ZengMilitary Health Department, Ministry of Defance, SerbiaVojnosanitetski Pregled0042-84502007-01-01641285986210.2298/VSP0712859TPrimary systemic amyloidosisTanasilović SrđanŽivanović DubravkaNikolić MilošTomović MajaElezović IvoMedenica LjiljanaBackground. 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. Case report. 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. Conclusion. 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.http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdfamyloidosissigns and symptomspurpuramacroglossiadiagnosisdrug therapytreatmentoutcome |
| spellingShingle | Tanasilović Srđan Živanović Dubravka Nikolić Miloš Tomović Maja Elezović Ivo Medenica Ljiljana Primary systemic amyloidosis Vojnosanitetski Pregled amyloidosis signs and symptoms purpura macroglossia diagnosis drug therapy treatment outcome |
| title | Primary systemic amyloidosis |
| title_full | Primary systemic amyloidosis |
| title_fullStr | Primary systemic amyloidosis |
| title_full_unstemmed | Primary systemic amyloidosis |
| title_short | Primary systemic amyloidosis |
| title_sort | primary systemic amyloidosis |
| topic | amyloidosis signs and symptoms purpura macroglossia diagnosis drug therapy treatment outcome |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdf |
| work_keys_str_mv | AT tanasilovicsrđan primarysystemicamyloidosis AT zivanovicdubravka primarysystemicamyloidosis AT nikolicmilos primarysystemicamyloidosis AT tomovicmaja primarysystemicamyloidosis AT elezovicivo primarysystemicamyloidosis AT medenicaljiljana primarysystemicamyloidosis |