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Juvenile Dermatomyositis with Gottron Lesions and a Scleroderma-Like Pattern

Name: Juvenile Dermatomyositis with Gottron Lesions and a Scleroderma-Like Pattern
Creator: Clara Udaondo

Juvenile DermatomyositisLate Scleroderma-Like PatternSevere

A 13-year-old girl with Gottron lesions, no muscle weakness, normal CPK, and a markedly pathological capillaroscopy at disease onset.

AuthorClara Udaondo

Clinical context

The patient was referred to rheumatology because of skin lesions compatible with juvenile dermatomyositis. Examination showed Gottron papules on the fingers and Gottron signs on the ankles, elbows, and knees. She had no muscle weakness, with an MMT score of 80/80. Laboratory testing showed normal CPK, positive ANA, and weak anti-PM/Scl positivity, with the remaining autoantibodies negative. Treatment was started with hydroxychloroquine and intravenous immunoglobulin.

Capillaroscopy

The baseline capillaroscopy is compatible with juvenile dermatomyositis and shows a severe scleroderma-like microangiopathy, with marked capillary loss, frequent enlarged and giant capillaries, hemorrhages, abnormal or arborizing shapes, and architectural disorganization.

Key Capillaroscopic Findings

Marked capillary loss (4.46/mm)
Frequent giant capillaries (21.1%) and enlarged capillaries (64.8%)
Hemorrhages and abnormal/arborizing capillaries in an active myositis-associated pattern

Juvenile Dermatomyositis with Gottron Lesions and a Scleroderma-Like Pattern