Anti-TIF-1γ Positive Juvenile Dermatomyositis Diagnosed with Atopic Dermatitis

Excerpt

A 7-y-old girl had cutaneous symptoms with pruritus and was diagnosed with atopic dermatitis (AD) one year before. Topical corticosteroids did not improve her cutaneous symptoms. Three months before, she experienced difficulty in crouching down. Physical examination revealed dry skin throughout the body (Fig. 1a), and erythematous areas on the flexor side of the bilateral knee joint, flexor side of the bilateral elbow joint, and neck, indicating AD (Fig. 1b–d), and erythematous areas on the upper back and eyelids and keratotic papules on the extensor surfaces of the finger joints, indicating shawl sign, heliotrope eruption, and Gottron sign, respectively (Fig. 1e–g). Dermoscopy revealed telangiectasia and meandering of the posterior nailfold (Fig. 1h). Blood examination revealed WBCs 9,600/µL; eosinophils 288/µL (3.0%); Creatine kinase (CK) 31 IU/L; aldolase, 15.8 mg/dL; IgE, 1,265 IU/mL; and thymus and activation-regulated chemokine (TARC) 701 pg/mL (<763 pg/mL). Anti-transcription intermediary factor 1γ (TIF1γ) autoantibodies were positive. Thus, she was diagnosed with TIF1γ-positive juvenile dermatomyositis (JDM). The cutaneous symptoms improved after treatment with corticosteroids and tacrolimus. …