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.
"Primary cutaneous amyloidosis is a local amyloidosis in which amyloid deposits are restricted to the skin. This disease has been classified into three types: macular amyloidosis, lichen amyloidosis, and nodular amyloidosis (Bhat et al., 2009; Schwendiman et al., 2009). Primary cutaneous nodular amyloidosis is thought to be a plasma cell dyscrasia, where amyloid light chain is involved and diagnosis is based on the absence of clonal plasma cell proliferation in the bone marrow; however some patients thought to be afflicted with only a localized version of this disease on long-term follow-up did indeed develop systemic amyloidosis. "
[Show abstract][Hide abstract] ABSTRACT: Amyloidosis are a large group of conformational diseases characterized by abnormal protein folding and assembly which results in the accumulation of insoluble protein aggregates that may accumulate systemically or locally in certain organs or tissue. In local amyloidosis, amyloid deposits are restricted to a particular organ or tissue. Alzheimer's, Parkinson's disease, and amyotrophic lateral sclerosis are some examples of neurodegenerative amyloidosis. Local manifestation of protein aggregation in the skin has also been reported. Brain and skin are highly connected at a physiological and pathological level. Recently several studies demonstrated a strong connection between brain and skin in different amyloid diseases. In the present review, we discuss the relevance of the "brain-skin connection" in different neurodegenerative amyloidosis, not only at the pathological level, but also as a strategy for the treatment of these diseases.
Frontiers in Neurology 01/2012; 3:5. DOI:10.3389/fneur.2012.00005
[Show abstract][Hide abstract] 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. · 0.60 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Please cite this paper as: Therapeutic removal of amyloid deposits in cutaneous amyloidosis by localised intra-lesional injections of anti-amyloid antibodies. Experimental Dermatology 2010; 19: 904–911.
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.
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