To the Editor: We read with great interest Narayan and Richardson’s report of dermatomyositis (DM) with multiple myositis-specific autoantibodies (MSAs), including anti-transcriptional intermediary factor 1-γ (TIF1-γ), SUMO-activating enzyme, and nuclear helicase protein-2.1 Other known dermatomyositis-specific MSAs (DM-MSAs) include anti-nuclear matrix protein-2 (NXP-2) and melanoma differentiation-associated gene-5 (MDA-5). Multiple DM-MSAs are uncommon, with an estimated prevalence of 15.7% among MSA-positive patients.2 Herein, we present a DM patient with TIF1-γ, MDA-5, and NXP-2 autoantibodies.
A 30-year-old woman presented with a 1-month history of a heliotrope rash (Fig 1, A), Gottron sign (Fig 1, B), V-neck erythema (Fig 1, C), hypopigmented, red-on-white patches (Fig 1, C and D), and muscle weakness. Laboratory studies revealed elevated creatine kinase (11,484; normal range, 5-150 U/L) and aldolase (29.4; normal range, 3.3-10.3 U/L). The extranuclear antigen antibody panel showed antiribonucleoprotein positivity. The Myomarker 3 Panel was also positive for anti-histidyl-tRNA synthetase, TIF1-γ, MDA-5, NXP-2, U1-ribonucleoprotein, and Sjögren-syndrome-related antigen A 52 antibodies. A punch biopsy revealed perivascular and interstitial dermatitis with interface changes and pigment-laden macrophages (Fig 2, A-C). Malignancy and interstitial lung disease screenings were unremarkable.
Fig 1.
Clinical features of the DM patient with TIF1-γ, MDA-5, and NXP-2 autoantibodies, including (A) heliotrope rash, (B) Gottron sign on the distal and proximal interphalangeal joints with frayed cuticles and periungual telangiectasia, (C) V-neck erythema and dyspigmented plaque, and (D) hypopigmented and telangiectatic (“red-on-white”) patches. DM, Dermatomyositis; MDA-5, melanoma differentiation-associated gene-5; NXP-2, nuclear matrix protein-2; TIF1-γ, transcriptional intermediary factor 1-γ.
Fig 2.
A punch biopsy from the back showed a sparse superficial perivascular lymphocytic infiltrate (A) H&E, ×4, with subtle hints of vacuolar degeneration and dermal melanin deposition (B) H&E, ×20. An Alcian blue stain highlighted foci of mucin deposition within the dermis (C) H&E, ×20. H&E, Hematoxylin and eosin.
Our case is notable for the presence of 6 distinct MSAs, including 3 DM-MSAs, 2 of which (NXP-2 and TIF1-γ) are associated with malignancy.3 The absence of vasculopathic ulcerations typically seen in the MDA-5 subtype and extensive hypopigmented and red-on-white telangiectatic patches suggests that our patient had the dominant TIF1-γ phenotype.4,5 This additional case of multiple positive DM-MSAs reinforces the importance of the clinical examination when evaluating DM patients.
Conflicts of interest
None disclosed.
Footnotes
Funding sources: None.
Patient consent: The authors attest that they have obtained written consent from patient/s, their legal guardian/s or person/s with legal authority, for their photographs and medical information to be published in print and online and with the understanding that this information may be publicly available. Patient consent forms were not provided to the journal but are retained by the authors to be made available upon request.
IRB approval status: Not applicable.
Data sharing and data availability: Data sharing is not applicable to this article as no datasets were generated or analyzed during the present study.
References
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