Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis

Abstract

Background: Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment.

Objective: We sought to define the clinical phenotype of patients with anti-TIF-1γ DM.

Methods: Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features.

Results: In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic ("red on white") patches.

Limitations: This was a retrospective study from a single tertiary referral center.

Conclusion: TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM.

Keywords: Cutaneous Dermatomyositis Assessment and Severity Index; autoantibodies; dermatomyositis; malignancy; phenotype; transcriptional intermediary factor-1γ.

Fig. 1

A, Psoriasiform plaque from a patient with dermatomyositis (DM) and anti-transcriptional intermediary factor (TIF)-1γ antibodies but no malignancy. Well-demarcated, erythematous plaque with hyperkeratotic scale on the abdomen. B, Hyperkeratotic Gottron papules from a patient with DM and anti-TIF-1γ antibodies. Thick plaques with micaceous scale over the joints of the back of hand. C, “Red on white” patch seen in a patient with DM. Hypopigmented patch on the forehead with intervening, follicular, erythematous, and telangiectatic macules. This patient was found to have anti-TIF-1γ antibodies but no malignancy. D, Hyperkeratotic papules on the palms from a patient with DM and anti-TIF-1γ antibodies. Hyperkeratotic and verrucous papules in a background of blanchable reticulated erythematous macules on the index finger without associated internal malignancy.