Literature DB >> 24383741

Pansclerotic morphea with features of eosinophilic fasciitis: distinct entities or part of a continuum?

Ashika Odhav1, Mark F Hoeltzel, Kristi Canty.   

Abstract

Scleroderma is a highly complex disorder in its clinical manifestations and pathogenesis. It has a wide range of clinical manifestations due to varying degrees of vasculopathy, autoimmunity, altered endothelium function, and abnormal fibrosis. The most widely used classification system grouped eosinophilic fasciitis and disabling pansclerotic morphea of childhood into the category of deep morphea. This previous classification does not include a category for overlapping conditions. A proposed new classification includes a new mixed subtype in which a combination of two or more of the previous subtypes is present in the same individual, although eosinophilic fasciitis has been excluded. We present the case of a 4-year-old boy who presented with features of disabling pansclerotic morphea and eosinophilic fasciitis simultaneously, which to our knowledge has not been previously reported. This suggests that these diseases are part of a more closely related continuum rather than separate disorders, as currently classified.
© 2014 Wiley Periodicals, Inc.

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Year:  2014        PMID: 24383741     DOI: 10.1111/pde.12279

Source DB:  PubMed          Journal:  Pediatr Dermatol        ISSN: 0736-8046            Impact factor:   1.588


  2 in total

Review 1.  Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.

Authors:  Han Jie Soh; Courtney Samuel; Victoria Heaton; William Douglas Renton; Angela Cox; Jane Munro
Journal:  Rheumatol Int       Date:  2019-03-05       Impact factor: 2.631

2.  Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature.

Authors:  Angie Christensen; Christina Di Loreto; Edward Smitaman; Taraneh Paravar; Karra A Jones; Monica Guma
Journal:  Clin Case Rep       Date:  2018-06-04
  2 in total

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