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Juvenile Dermatomyositis anti-TIF1 with Active Scleroderma-Like Pattern

Name: Juvenile Dermatomyositis anti-TIF1 with Active Scleroderma-Like Pattern
Creator: Clara Udaondo

Juvenile Dermatomyositis (anti-TIF1+)Active Scleroderma-Like PatternSevere

A 12-year-old girl with Gottron lesions, facial erythema, muscle weakness, dysphagia, and anti-TIF1 antibodies.

AuthorClara Udaondo

Clinical context

The patient was referred for skin lesions, including Gottron lesions and facial erythema, together with muscle weakness and dysphagia. Complementary tests were compatible with juvenile dermatomyositis, and anti-TIF1 antibodies were positive.

Capillaroscopy

Capillaroscopy shows a severe active scleroderma-like pattern, with very low capillary density, numerous giant capillaries, frequent enlarged capillaries, arborizing abnormal shapes, and architectural disorganization. This morphology is highly suggestive of active microangiopathy in juvenile dermatomyositis.

Key Capillaroscopic Findings

Severe capillary loss (2.89/mm)
Very frequent giant capillaries (44.6%)
Abnormal/arborizing capillaries and marked architectural disorganization

Juvenile Dermatomyositis anti-TIF1 with Active Scleroderma-Like Pattern