Case Report: Mycosis Fungoides in Dermatomyositis

Despite its rarity, cutaneous T cell lymphoma should be considered in DM patients presenting with such skin findings as atypical plaques, nodules, ulcerations & tumor formation.

Screening guidelines for DM patients are needed for practicing rheumatologists. In a cohort of DM patients, it was found that age-appropriate cancer screening is not aggressive enough. In addition to your usual malignancy screening, computerized tomography of the chest, abdomen and pelvis also aid in a malignancy diagnosis.⁷ Features that should prompt investigation for malignancy include refractoriness to treatment, older age, leukocytoclastic vasculitis, severe muscle disease, low C4, cytopenias and hyperimmunoglobulinemia.³

Certain myositis-specific antibodies, such as anti-nuclear matrix protein-2 (anti-NXP-2) antibody and anti-transcriptional intermediary factor 1 γ (anti-TIF1γ) antibody, are associated with malignancy, but anti-Mi-2 antibodies are not. In a large cohort of malignancy-associated DM, 83% of patients were found with either anti-TIF1γ or anti-NXP-2 antibodies. Patients with either antibody had an increased risk of developing malignancy, with an odds ratio of 3.8.¹¹ Anti-Mi-2 antibodies are associated with classic cutaneous DM, lower interstitial lung disease risk, milder muscle disease and good treatment response. Anti-NXP-2 antibodies are associated with juvenile DM, calcinosis and severe disease. Anti-TIF1γ antibodies are associated with early diagnosis of malignancy, severe cutaneous disease and ulcerations.¹² 

Our case highlights the importance of heightened surveillance in DM patients despite the type of biomarkers present.¹²

Barrett Ford, MD, is a fellow in the Section of Rheumatology at the Louisiana State University Health Sciences Center, New Orleans.

Chandana Shilpa Kollipara Ravipati, DO, is an assistant professor of clinical medicine in the Section of Rheumatology at Louisiana State University Health Sciences Center.

Nirupa Patel, MD, is an associate professor of clinical medicine in the Section of Rheumatology at the Louisiana State University Health Sciences Center.

References

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