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Anti-NXP2-Positive Paraneoplastic Dermatomyositis With Histopathologic Changes Confined to the Acrosyringia.
- Source :
-
The American Journal of dermatopathology [Am J Dermatopathol] 2017 Jan; Vol. 39 (1), pp. e3-e7. - Publication Year :
- 2017
-
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.<br />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.<br />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.<br />Conclusion: 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.
- Subjects :
- Adrenal Cortex Hormones therapeutic use
Aged
Biomarkers analysis
Biopsy
Carcinoma, Non-Small-Cell Lung pathology
Carcinoma, Non-Small-Cell Lung therapy
Dermatomyositis drug therapy
Dermatomyositis etiology
Dermatomyositis pathology
Humans
Immunoassay
Lung Neoplasms pathology
Lung Neoplasms therapy
Male
Paraneoplastic Syndromes drug therapy
Paraneoplastic Syndromes etiology
Paraneoplastic Syndromes pathology
Skin drug effects
Skin pathology
Tomography, X-Ray Computed
Treatment Outcome
Adenosine Triphosphatases immunology
Autoantibodies analysis
Carcinoma, Non-Small-Cell Lung complications
DNA-Binding Proteins immunology
Dermatomyositis immunology
Lung Neoplasms complications
Paraneoplastic Syndromes immunology
Skin immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1533-0311
- Volume :
- 39
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- The American Journal of dermatopathology
- Publication Type :
- Academic Journal
- Accession number :
- 28045754
- Full Text :
- https://doi.org/10.1097/DAD.0000000000000694