there are basically three variants of primary localized cutaneous amyloidosis ( plca ) : macular , papular ( lichenoid ) and nodular forms [ 1 , 2 , 3 ] . in macular and papular plca , cytokeratins serve as the amyloid precursors , and these variants are limited to the dermis [ 1 , 2 , 3 , 4 ] . in contrast , the rare type of nodular plca originates from immunoglobulin light chains ; it may extend to the subcutaneous tissue , and blood vessel infiltration can also be observed [ 1 , 2 , 3 , 5 ] . furthermore , nodular plca can originate from systemic amyloidoses or progress to systemic disease . here , we present a rare case of nodular plca , which resembled a giant tumor on the head . a 79-year - old man presented with a large tumor on the left side of his head ( fig . the waxy tumor exhibited a sulcated , mostly yellowish and reddish surface , and in some areas hemorrhages . a punch biopsy showed amorphous eosinophilic material in the dermis , so that we suspected a giant amyloid tumor [ 1 , 2 , 4 ] . histology showed that nearly the entire dermis was filled with amorphous eosinophilic material ( fig . immunohistochemistry was negative for cytokeratin , but showed that the entire dermis and parts of the subcutis were filled with amyloid consisting of both kappa and lambda immunoglobulin light chains ( fig . , we performed fine - needle abdominal fat aspiration , and the samples showed only minimal traces of amyloid , thus no signs of systemic amyloidosis . therefore , the diagnosis was primary localized cutaneous nodular light chain amyloidosis . at 1-year follow - up the diagnosis of plca requires histological analysis of a skin specimen . in order to discriminate the amyloid precursors in plca , immunohistochemistry with antibodies against cytokeratin and immunoglobulin light chains ( lambda and kappa subtypes ) it is best to also use electron microscopic work - up of a sample fixed in karnofsky 's fixative ( glutaraldehyde ) . as amyloid fibrils are quite stable , electron microscopy can also be done from formalin - fixed samples ; however , image quality will be reduced with this standard fixative . the typical amyloid fibrils with diameters of 710 nodular plca can often be successfully treated surgically , e.g. via curettage , excision or ablative laser therapy ( co2 lasers etc . ) . patients should regularly be screened for progression to systemic disease as this may occur in 550% of cases . to do so , a full history and physical examination along with an electrocardiogram , complete blood count , serum creatinine level , serum liverassociated enzymes levels , serum protein electrophoresis and urine protein electrophoresis should be preformed . it has also been suggested that an abdominal fat biopsy ( easy access for screening ) be performed to rule out systemic disease . to our knowledge , this is the most extensive case of nodular plca reported in the literature .