Primary cutaneous nodular amyloidosis: case report and review of the literature.
ABSTRACT Primary cutaneous nodular amyloidosis (PCNA) is a rare form of primary cutaneous amyloidosis. It presents as waxy yellow-red nodules that are located preferentially on the lower extremities, face, scalp, and genitals. Recognition of this condition is of particular importance, as primary systemic amyloidosis can have a similar cutaneous presentation. We report a case of PCNA in a 52-year-old woman with systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). We discuss the need to evaluate for systemic disease and provide a concise review of the literature focusing on clinical presentation, disease associations, and management.
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ABSTRACT: We report an unusual case of a 76-year-old woman with primary cutaneous amyloidosis who initially presented with features of asteatotic eczema that was unresponsive to topical corticosteroid treatment. Histological examination revealed amyloid deposits involving the superficial and deep dermis. These lesions later gradually evolved into erythematous nodules, and a second biopsy performed 29 months after the initial presentation again revealed diffuse collections of amyloid throughout the dermis. Further investigations did not reveal evidence of systemic involvement, thus indicating a diagnosis of primary cutaneous nodular amyloidosis. The initial presentation as eczematous lesions illustrates the importance of clinicopathological correlation and subsequent follow-up.Singapore medical journal 09/2010; 51(9):e158-60. · 0.63 Impact Factor
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ABSTRACT: In the skin, amyloidosis can be found with or without systemic disease. Primary cutaneous amyloidosis defines those amyloidoses restricted to the skin without involvement of other systems. Here, we used conformation-specific antibodies to characterise both fibrillar and oligomeric amyloid aggregates in the skin from patients with cutaneous amyloidosis. Localised cutaneous amyloidosis with different morphology was reproduced in mice by intra-dermal (i.d.) and subdermal administration of amyloid-enhancing factor. Moreover, we demonstrated that conformational antibodies were effective in clearing amyloid deposits caused by localised intra-lesional injections without the necessity of an immune response. Given the accessibility and amyloid localization in this disease, direct i.d. injections of conformational antibodies could be a convenient and direct method for treatment.Experimental Dermatology 10/2010; 19(10):904-11. DOI:10.1111/j.1600-0625.2010.01121.x · 4.12 Impact Factor
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ABSTRACT: Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. Typically presenting as waxy nodules on the lower extremities, it demonstrates localized deposition of AL-type amyloid in immunohistologic study and is often associated with focal plasma cell proliferation. Sjögren syndrome, an autoimmune lymphoproliferative disorder, is characterized by keratoconjunctivitis sicca and xerostomia with lymphocytic infiltration of exocrine glands. As shown in case reports, the association of LCNA with Sjögren syndrome is considerable. Herein, we report a 78-year-old woman with LCNA, who was further surveyed and diagnosed with Sjögren syndrome. In light of the significant relation between these 2 diseases, further examination for coexistence of Sjögren syndrome in addition to systemic amyloidosis is well warranted. Prompt identification of an underlying Sjögren syndrome in LCNA with polyclonal immunoglobulin amyloid may have important therapeutic consequences.Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases 10/2011; 17(7):368-70. DOI:10.1097/RHU.0b013e31823209ba · 1.25 Impact Factor