Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.
Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.
Summers
Erika M
EM
Department of Internal Medicine, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA. esummers@bidmc.harvard.edu
Kendrick
Christina G
CG
D000686:Amyloidosis / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D017675:CREST Syndrome / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008875:Middle Aged; D012875:Skin Diseases, Metabolic / Q000150:complications* / Q000473:pathology* / Q000628:therapy