Maria Syed*, Afaq Ahmad* and Anwar Ul Haque**
*Department of Dermatology. Pakistan Institute of Medical Sciences, Islamabad, Pakistan.
**Department of Pathology AJK Medical College, AJK University, Muzaffarabad, Azad Kashmir
Abstract: Primary systemic amyloidosis is a rare entity. We report a case of primary systemic amyloidosis without
any associated hematological abnormality who presented with macroglossia and hemorrhagic lesions around
eyes and sub mammary region. There were soft lobulated masses around perianal region, labia majora and on external
auditory meatus obstructing auditory canal. She was also operated for carpel tunnel syndrome in thepast.
Urine and serum electrophoresis were negative. Diagnosis was confirmed by biopsy of submammary skin lesion
using haematoxylin and eosin and Congo red stain. Polarized microscopy was used to see birefringence of amyloid.
Key words: Amyloidosis, Primary systemic amyloidosis, macroglossia, carpal tunnel syndrome, electrophoresis.