A 40 year old female patient presented with myalgia and both knee joint pain for 3 years and raised skin lesions over both eyelids for 1 year. General examination and systemic examination were normal and vitals were stable. Dermatological examination showed multiple well defined erythematous smooth plaques over both eyelids, waxy papules over upper lips, microstomia, macroglossia, sclerodactyly, nodules over both suprascapular region, fixed flexion contracture of index and middle finger present. Routine investigations were normal.  Serum protein electrophoresis showed band in gamma globulin region. Histopathology showed homogenous eosinophilic material in papillary dermis and dense fibrocollagenous tissue in reticular dermis. Congo red stain was positive. Hence diagnosis of primary systemic amyloidosis was made.

Keywords: Sclerodactyly, macroglossia, systemic amyloidosis