Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide |
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| Authors: | Philip L Tong William A Walker Ross J Glancy Julian P Cooney Kurt Gebauer |
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| Affiliation: | 1. Sir Charles Gairdner Hospital;2. Murdoch University School of Nursing;3. Murray Medical Centre, Mandurah;4. University of Notre Dame School of Medicine, Fremantle, Western Australia;5. Department of Histopathology, Fremantle Hospital;6. School of Pathology and Laboratory Medicine;7. School of Medicine and Pharmacology, University of Western Australia;8. Haematology Department, Royal Perth Hospital, Perth;9. Fremantle Dermatology, and |
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| Abstract: | Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare subtype of localized cutaneous amyloidosis and can be associated with various connective tissue disorders. It can be difficult to treat and past therapies include surgical excision, dermabrasion, electrodessication and curettage, cryotherapy and laser therapy. We present a case of a middle‐aged woman with PLCNA associated with CREST (calcinosis, Raynaud phenomenon, oesophageal motility disorders, sclerodactyly and telangiectasia) syndrome and Sjögren's syndrome responding to cyclophosphamide with no new amyloid deposits and resolution of skin ulceration after many years of resistance to drug therapy. It is important to monitor these patients for progression into systemic amyloidosis. |
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| Keywords: | amyloid chemotherapy CREST cyclophosphamide nodular amyloidosis primary localized cutaneous amyloidosis Sjö gren's syndrome treatment |
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