On the occasion of the diagnosis of a case of autoimmune necrotizing myopathy (AINM) in one of their patients, two Japanese physicians provide an update on this infrequent and therefore poorly understood pathology in paediatrics:
- an exhaustive and critical analysis of the literature enabled them to identify 33 cases of AINM in patients under the age of 18,
- Almost all of these patients had a CPK level above 5000 IU/L,
- 44% of them had rash-like skin signs, especially in those over seven years old,
- Most often, the rash was erythematous
- the definitive diagnosis was based on the positivity of anti-HMGCR autoantibodies.
The authors emphasise the importance of skin signs as an element of orientation for the diagnosis of AINM.
