title: Nodular Scleroderma Revisited: Systemic Sclerosis Presenting as Annular Keloidal Sclerotic Plaques.
abstract: BACKGROUND
  Nodular scleroderma, also known as keloidal scleroderma, is a rare variant of systemic sclerosis.
  
  
  OBJECTIVE
  The clinical features, pathologic findings and postulated pathogenesis of nodular scleroderma are discussed.
  
  
  METHODS
  A woman with previously undiagnosed systemic sclerosis who presented with nodular scleroderma is described. Using the PubMed database, a literature search was performed on keloidal scleroderma, nodular scleroderma, and systemic sclerosis.
  
  
  RESULTS
  Nodular scleroderma is characterized by firm plaques or nodules, which can mimic a keloid, that are typically located on the anterior orposterior upper trunk and the arms; they show pathologic changes of scleroderma, keloid, or hypertrophic scar. Akeloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis, in individuals who are genetically predisposed to keloid formation, is the hypothesized pathogenesis.
  
  
  CONCLUSIONS
  Nodular scleroderma is rare. The authors' patient presented with diarrhea, dysphagia, fatigue, Raynaud's phenomenon, shortness of breath, and annular keloidal plaques of morphea whose biopsy showed features of hypertrophic scar; additional studies confirmed the diagnosis of the nodular scleroderma variant of systemic sclerosis. The possibility of systemic sclerosis should be entertained in patients who present with nodularor keloidal plaques that morphologically resemble morphea and have histologic findings of a scar or a keloid-especially if there are associated symptoms suggestive for systemic sclerosis.
authors.affiliation: The Division of Dermatology, University of California San Diego, San Diego, California;
authors.forename: David N
authors.initials: DN
authors.lastname: Lortscher
authors.affiliation: The Division of Dermatology, University of California San Diego, San Diego, California;
authors.forename: Philip R
authors.initials: PR
authors.lastname: Cohen
authors.affiliation: The Department of Dermatology, University of Texas Health Science Center at Houston, Houston, Texas.
authors.forename: Carolyn A
authors.initials: CA
authors.lastname: Bangert
authors.affiliation: The Division of Dermatology, University of California San Diego, San Diego, California;
authors.forename: Taraneh
authors.initials: T
authors.lastname: Paravar