CASE REPORTS
JOURNAL ARTICLE
Add like
Add dislike
Add to saved papers

[Juvenile dermatomyositis: Early onset and unusual presentation].

Juvenile dermatomyositis (JDM) is a rare disease, with a mean age of onset of 7 years. We report a case of JDM in a 13-month-old infant.

OBSERVATION: A 13-month-old infant presented with an edema of the upper lip, 4 days after receiving amoxicillin-clavulanate. The patient was treated with betamethasone and an antihistamine. Progression was marked by the appearance of a white edema of the periorbital area and the upper lip, with purpuric lesions, aphthoid ulcerations, and a drooping head. Very high muscle enzymes, a myogenic electromyogram, and hypertrophic cardiomyopathy were found and diagnosis of JDM was retained. The patient was treated with a high dose of corticosteroids and methotrexate. Before the installation of dysphagia, dysphonia, and axial hypotonia, the bolus of cyclophosphamide was administered, with clear improvement. Corticosteroids and methotrexate were then kept over the long term.

DISCUSSION AND CONCLUSION: The originality of this observation of JDM resides in the very young age of onset of the disease, its unusual cutaneous signs including labial edema extending to the regions around the eyes, its severe muscle manifestations causing axial hypotonia, and finally, in its association with hypertrophic cardiomyopathy. Early initiation of treatment (12 days after the onset of symptoms) greatly improved the prognosis of this form of JDM, which is very severe and refractory to first-line therapy.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app