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.
- SourceAvailable from: ncbi.nlm.nih.gov[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.
- [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. · 0.63 Impact Factor