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Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide
Author(s) -
Tong Philip L,
Walker William A,
Glancy Ross J,
Cooney Julian P,
Gebauer Kurt
Publication year - 2013
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/j.1440-0960.2011.00770.x
Subject(s) - medicine , sclerodactyly , amyloidosis , dermatology , crest syndrome , calcinosis , cryotherapy , pathology , poikiloderma , hypopigmentation , connective tissue disease , surgery , calcification , disease , autoimmune disease
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.