A and B, Clinical images from 2 patients, both showing the typical arcuate symmetric erythema on the hard palate intermixed with white macules. C-E, Biopsy specimens from an ovoid palatal patch showing an interface dermatitis with dyskeratotic keratinocytes (C, original magnification ×20), a markedly thickened basement membrane (D, original magnification ×40), and increased dermal mucin (E, original magnification ×40).
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Bernet LL, Lewis MA, Rieger KE, Casciola-Rosen L, Fiorentino DF. Ovoid Palatal Patch in Dermatomyositis: A Novel Finding Associated With Anti-TIF1γ (p155) Antibodies. JAMA Dermatol. 2016;152(9):1049–1051. doi:10.1001/jamadermatol.2016.1429
Dermatomyositis (DM) is a heterogeneous disease with a multitude of physical findings and clinical presentations, and patients with anti–transcriptional intermediary factor-1γ (TIF1γ) antibodies have distinct cutaneous features and are also at increased risk for cancer.1 We describe a novel, distinctive patch on the hard palate, which is associated with anti-TIF1γ antibodies that may identify patients at higher risk of cancer.
The Stanford University School of Medicine institutional review board approved this study, and all patients provided written informed consent for participation. All consecutive patients seen in the Stanford dermatology clinics between November 2014 and March 2015 and who met the Bohan and Peter2 or Sontheimer (amyopathic patients)3 criteria for DM were included. We recorded demographic and historical clinical data, a Cutaneous Disease Activity Score Index Activity (CDASI-a) score, and oral findings. Clinically amyopathic and cancer-associated DM were defined as previously described.1 The 2-tailed Fisher exact test and Mann-Whitney test were used to determine P values for dichotomous and continuous variables, respectively.
Data were recorded for 52 consecutive patients, of whom 45 were included in the analysis (Table). Six were excluded owing to a lack of antibody data, and 1 owing to a prior diagnosis of oral lichen planus. Eighteen (40%) of the 45 patients had a well-demarcated, erythematous patch on the posterior hard palate. This patch did not ulcerate, often contained white macular markings and a symmetric arcuate configuration across the midline, and was asymptomatic (Figure, A and B). Biopsy of 1 lesion revealed an interface dermatitis with a thickened basement membrane and increased dermal mucin (Figure, C-E).
We next tested if this finding was associated with any clinical or laboratory features; these findings are quantitatively detailed in the Table. Briefly, the patch was significantly associated with the presence of an anti-TIF1γ antibody (P < .001). None of the 16 patients with any of the other defined antibodies had this oral lesion. The oral lesion was associated with female sex (P = .01) and clinically amyopathic disease (P = .03). Surprisingly, the ovoid patch was also highly associated with cancer-associated DM (P = .004); in fact, of the 6 anti-TIF1γ antibody–positive patients with cancer, all 6 had this oral lesion. Of the 7 patients with an ovoid patch and cancer, 6 were anti-TIF1γ antibody positive. There was no association with the presence of antinuclear antibody, interstitial lung disease, CDASI-a score, ethnicity, age, or disease duration.
Knowledge of the oral manifestations of DM has been limited to small series or case reports. Associated findings include lichen planus, ulcerations, gingival telangiectasia and erythema, gingival vasculopathy, and desquamative gingivitis.4,5 We describe a novel hard palate lesion in DM that we term the ovoid palatal patch and that is highly associated with the presence of anti-TIF1γ antibodies. Interestingly, this patch may identify patients with cancer in the anti-TIF1γ antibody–positive population.
The small sample size and the enrichment of anti-TIF1γ antibody–positive patients in our cohort may have limited our sensitivity for detecting this finding in the other antibody subtypes, as well as our ability to determine if the cancer association may be with the anti-TIF1γ antibody alone. However, it is interesting that, in our cohort, the cancer risk in patients with and without anti-TIF1g antibodies does not meet statistical significance (6 of 23 and 2 of 22, respectively; P = .24).1 Despite these limitations, we call attention to a novel, easily identifiable clinical finding in DM that is found in 40% of patients. It will be interesting to test if this finding and its characteristic location and shape distinguishes DM from other clinical mimickers (such as lupus erythematosus). Larger studies are needed to confirm the specificity of this finding and its sensitivity for predicting other outcomes such as cancer or amyopathic disease.
Corresponding Author: David F. Fiorentino, MD, PhD, Department of Dermatology, Stanford University School of Medicine, 450 Broadway, Pavilion C, C-234, Redwood City, CA 94063 (email@example.com).
Accepted for Publication: April 10, 2016.
Published Online: May 25, 2016. doi:10.1001/jamadermatol.2016.1429.
Author Contributions: Drs Bernet and Fiorentino had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: Bernet, Lewis, Fiorentino.
Acquisition, analysis, or interpretation of data: Bernet, Lewis, Rieger, Casciola-Rosen, Fiorentino.
Drafting of the manuscript: Bernet.
Critical revision of the manuscript for important intellectual content: Bernet, Lewis, Rieger, Casciola-Rosen, Fiorentino.
Statistical analysis: Bernet, Fiorentino.
Administrative, technical, or material support: Lewis, Rieger, Casciola-Rosen.
Study supervision: Lewis, Fiorentino.
Conflict of Interest Disclosures: None reported.
Funding/Support: The Johns Hopkins Rheumatic Disease Research Core Center, where the antibody assays were performed, is supported by the National Institutes of Health (NIH) grant P30-AR-053503.
Role of the Funder/Sponsor: The NIH had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
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