Anti-TIF1gamma Antibody-Positive Dermatomyositis Associated with Myelodysplastic Syndrome: Response to Treatment

Abstract

Dermatomyositis (DM) classically presents as a dyad of typical cutaneous findings and varying degrees of proximal muscle weakness. Interestingly, the development of DM may signal underlying malignancy, and numerous myositis-specific autoantibodies have been associated with this paraneoplastic phenomenon. Positivity for anti-TIF1gamma antibody, in particular, raises suspicion for cancer-associated DM. Here, we present an unusual case of anti-TIF1gamma antibody-positive DM that ultimately lead to the diagnosis of myelodysplastic syndrome (MDS). Importantly, topical treatment and chemotherapy targeting MDS resulted in a swift and remarkable amelioration of cutaneous disease.

Keywords: dermatomyositis; myelodysplastic syndrome.

Figure 1. Dermatologic Exam

The patient presented with cutaneous features of dermatomyositis, including red-on-white poikiloderma of the scalp (A), violaceous erythema overlying upper eyelids (heliotrope rash), periorbital edema, photo-distributed poikiloderma with violaceous plaques (C), and periungual erythema and tenderness (E). Ten weeks after initiation of chemotherapy for myelodysplastic syndrome, along with only topical treatment, cutaneous symptoms of dermatomyositis were significantly improved (B, D, and F).

Figure 2. Magnetic Resonance Imaging (MRI)

Pelvic MRI showed mild increased short TI inversion recovery (STIR) signal in anterior thigh muscles bilaterally, indicated by red arrows. Findings were consistent with inflammatory myopathy.