Image 1-Amyloid nodules with characteristic cracking.

Image 2-Congo red stain showing red blush of amyloid.

Image 3-Characteristic apple-green birefringence with polarized light examination.

Case Study

This is a 64 year old woman with a long history of CREST syndrome. She presents with a mass lesion in her left leg of several years duration. She has noted a recent increase in size with some pain.

Diagnosis:

Amyloidoma arising in the setting of CREST syndrome

Comment:

This is a fascinating case, not only because of the unusual presentation of an amyloidoma as a soft tissue mass, but also because of the rare association with CREST syndrome. A medical literature search could only find one other association with scleroderma.

The Congo Red stain is impressive even without polarization but does show the characteristic apple-green birefringent staining pattern, confirming the diagnosis.

References:

The Doctor's Doctor Website-Scleroderma

Amyloid deposition associated with generalized morphea-like scleroderma.
Eur J Dermatol. 2003 Sep-Oct;13(5):509-11.

Soft tissue amyloidoma of the extremities: a case report and review of the literature.
Arch Pathol Lab Med. 2004 Nov;128(11):1270-3.