Primary systemic amyloidosis with malignant pinch purpura: A case report
Keywords:
primary systemic amyloidosis, pinch purpuraAbstract
Bilateral periorbital purpura (raccoon’s eyes) has been associated with many causes such as traumatic (basal skull fracture and rhinoplasty), neuroblastoma, lymphoma, kaposi’s sarcoma and primary systemic amyloidosis. We report 88-year-old man presented with a 6 months history of extensive periorbital hemorrhagic nodular plaques forming gross distortion which disturbed his eye-fields and numerous translucent and hyperpigment papules over trunk and back. Histopathologic study from the skin lesions at periorbital region and back showed amorphous eosinophilic material deposit in superficial to deep dermis and around blood vessel wall. After Congo red staining, the sample showed a red-green birefringence and dichroism under polarized light microscopy. Further work-up revealed free light chain (kappa) on urine protein electrophoresis and serum immunofixation electrophoresis showed IgG kappa monoclonal gammopathy. According to these findings, the diagnosis of primary systemic amyloidosis associated with plasma cell dyscrasia was made. In conclusion, this case presented with massive periorbital hemorrhagic nodular mass and numerous papular eruptions which were rarely seen in systemic amyloidosis. Malignant pinch purpura was shown as a crucial clue in the diagnosis of systemic amyloidosis in our patient who has no other systemic complaints.
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เนื้อหาและข้อมูลในบทความที่ลงตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นข้อคิดเห็นและความรับผิดชอบของผู้เขียนบทความโดยตรงซึ่งกองบรรณาธิการวารสาร ไม่จำเป็นต้องเห็นด้วย หรือร่วมรับผิดชอบใดๆ
บทความ ข้อมูล เนื้อหา รูปภาพ ฯลฯ ที่ได้รับการตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นลิขสิทธิ์ของวารสารฯ หากบุคคลหรือหน่วยงานใดต้องการนำทั้งหมดหรือส่วนหนึ่งส่วนใดไปเผยแพร่ต่อหรือเพื่อกระทำการใดๆ จะต้องได้รับอนุญาตเป็นลายลักอักษรจากบรรณาธิการวารสารโรคผิวหนังก่อนเท่านั้น
