Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature |
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Authors: | Summers Erika M Kendrick Christina G |
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Institution: | Department of Internal Medicine, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA. esummers@bidmc.harvard.edu |
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Abstract: | 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. |
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