Pansclerotic Morphea with Features of Eosinophilic Fasciitis: Distinct Entities or Part of a Continuum?

• Published in: Pediatric dermatology Vol. 31; no. 2; pp. e42 - e47
• Main Authors: Odhav, Ashika, Hoeltzel, Mark F., Canty, Kristi
• Format: Journal Article
• Language: English
• Published: United States 01.03.2014
• Subjects: Child, Preschool; Diagnosis, Differential; Drug Therapy, Combination; Eosinophilia - diagnosis; Eosinophilia - drug therapy; Eosinophilia - pathology; Fasciitis - diagnosis; Fasciitis - drug therapy; Fasciitis - pathology; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Male; Scleroderma, Localized - diagnosis; Scleroderma, Localized - drug therapy; Scleroderma, Localized - pathology
• ISSN: 0736-8046; 1525-1470
• DOI: 10.1111/pde.12279

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.