Article

Primary cutaneous nodular amyloidosis: case report and review of the literature.

Des Moines University College of Osteopathic Medicine and Surgery, Iowa, USA.
Cutis; cutaneous medicine for the practitioner (Impact Factor: 0.82). 08/2009; 84(2):87-92.
Source: PubMed

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.

0 Bookmarks
 · 
72 Views
  • Clinical and Experimental Dermatology 03/2013; 38(2):200-2. · 1.33 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Primary cutaneous amyloidosis describes a group of disorders in which amyloid is deposited in the skin without evidence of systemic involvement. Nodular localized primary cutaneous amyloidosis (NLPCA) is a rare form of these skin-restricted amyloidoses. We present an unusual case of NLPCA in a 51-year-old man, who had clinical and histopathological evidence of subepidermal bullous formation, a unique feature in NLPCA. The possible pathogenesis of this change is discussed.
    Clinical and Experimental Dermatology 04/2014; 39(3):344-7. · 1.33 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Cutaneous nodular amyloidosis is rare, and association with Sjögren's syndrome has been reported. We discuss the possible link between these two diseases based on a case we saw. A 78-year-old woman with Sjögren's syndrome ongoing for 17 years presented for evaluation of a waxy infiltrated lesion on her left leg that had appeared 6 months earlier. Histopathological examination revealed a deposit of homogenous eosinophilic material throughout the dermis consistent with amyloidosis. Immunohistochemical study showed these deposits to be AL immunoglobin light chains. The association of cutaneous nodular amyloidosis and Sjögren's syndrome appears to be a distinct disease entity reflecting the polymorphic clinical spectrum of lymphoproliferative diseases related to Sjögren's syndrome.
    Annales de Dermatologie et de Vénéréologie 05/2013; 140(5):378-81. · 0.60 Impact Factor