Skin Involvement in Primary Systemic Amyloidosis

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susheel kumar


Primary systemic amyloidosis, papules, skin lesions, macroglossia.


Primary systemic amyloidosis is a rare disease. It primarily involves kidney, heart, peripheral nerves and liver. Intracutaneous hemorrhage manifesting in the form of petechiae, purpura and ecchymoses due to infiltration of blood vessel walls by amyloid deposits are the most common skin lesions. We report a case of primary systemic amyloidosis with multiple, non-itchy, papular lesions in lower eyelids and lower chest wall bilaterally. Diagnosis was confirmed in this case by biopsy of skin lesions using congo red staining. Papular eruptions as seen in index patient are relatively uncommon form of skin manifestations.


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Figure 1. Photographs showing (a) enlarged tongue with (b) teeth marks over the lateral margin and (c, d) multiple, papular lesions over lower chest wall Downloads 0
Figure 2. Microphotograph showing pink eosinophilic homogenous material in the dermis, H&E X 20 Downloads 0
Figure 3. Pink eosinophilic material showing pale orange positivity with Congo red stain Downloads 0