title: Anti-NXP2-Positive Paraneoplastic Dermatomyositis With Histopathologic Changes Confined to the Acrosyringia.
abstract: BACKGROUND
  Paraneoplastic syndromes consist of a group of disorders that are not related to the extension of the primary tumor or its metastases and that might be the first manifestation of a hidden neoplasm. It is a well-known association between dermatomyositis (DM) and cancer, especially gynecological tumors in women and lung cancer in men.
  
  
  METHODS
  We describe the case of a 67-year-old male who developed muscular weakness and pruritic skin lesions. Skin biopsies were performed and histologic findings were consistent with DM.
  
  
  RESULTS
  Skin biopsy showed interface dermatitis with vacuolar degeneration of the basal layer, dermal mucin deposits, and necrotic keratinocytes in the acrosyringia, a finding that has been previously reported in lupus erythematous but not in DM. Autoimmunity tests showed positivity for antinuclear antibodies and anti-NXP2, a recently described antibody associated with juvenile DM and, more rarely, with paraneoplastic DM.
  
  
  CONCLUSIONS
  We present the first case in the literature with histopathologic changes of DM affecting the acrosyringia. Besides, our patient autoimmunity results support the utility of the new myositis-specific autoantibodies and its relation with a clinical phenotype.
authors.affiliation: Departments of*Dermatology, †Immunology, and ‡Pathology, Fundación Jimenez Diaz, Universidad Autónoma, Madrid, Spain.
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Ramírez-Bellver
authors.forename: Elena
authors.initials: E
authors.lastname: Macías
authors.forename: Claudia
authors.initials: C
authors.lastname: Bernárdez
authors.forename: Joaquín
authors.initials: J
authors.lastname: López-Robles
authors.forename: Maria Del Carmen
authors.initials: MD
authors.lastname: Vegas-Sánchez
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Díaz-Recuero
authors.forename: Hernán
authors.initials: H
authors.lastname: Quiceno
authors.forename: Luis
authors.initials: L
authors.lastname: Requena
mesh_terms: D000251:Adenosine Triphosphatases / Q000276:immunology*; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D002289:Carcinoma, Non-Small-Cell Lung / Q000150:complications* / Q000473:pathology / Q000628:therapy; D004268:DNA-Binding Proteins / Q000276:immunology*; D003882:Dermatomyositis / Q000188:drug therapy / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007118:Immunoassay; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D012867:Skin / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D015415:Biomarkers; D004268:DNA-Binding Proteins; D000251:Adenosine Triphosphatases; C551732:MORC3 protein, human