Juvenile Dermatomyositis: About a Case
Aichaoui H^*, Mekchouche H¹, Naceur M², Mahmoudi N³, Bouziane-Nedjadi K⁴

1,2,4. Department of Pediatrics “C” (A. Cabral) CHUO.

3. CHUO Dermatology Department.

*Corresponding Author: Dr. Aichaoui H *, Department of Pediatrics “C” (A. Cabral) CHUO.

Received Date:  October 16, 2020

Publication Date: November 01, 2020

Abstract

Dermatomyositis is a Connectivity’s, a chronic vascular inflammatory disease affecting the skin and the striated muscular system. It is very rare, affects 1 to 4 people per million.

The objective of our work: To make this pathology known through our patient.

Observation: 8-year-old boy referred for suspected rheumatic fever put on Extencilline every 21 days. he had arthralgias with stiffening especially in the morning on waking evolving for 6 months, affecting the large and small joints bilaterally and symmetrically, repeated mouth ulcers, a rash on the face, lower back, knees and hands, erythematous lesions on the face, red-purplish on the upper eyelids and the metacarpophalangeal and interphalangeal joints. The joints are painful when mobilized. electrical signs in favor of myogenic damage, the elevation of muscle enzymes (LDH, CPK, aldolase), and muscle testing showing damage to the muscles of the neck and trunk. the diagnosis of dermatomyositis is obvious. Corticosteroid therapy initiated at a high dose then combined with methotrexate.

Conclusion: Dermatomyositis is very rare, the clinical picture of which is often confused with other Connectivity’s.

Keywords: Dermatomyositis, Juvenile, Connectivity’s, Rare, Pediatrics, Inflammatory, Vascular, Muscle.

Introduction

Dermatomyositis is a connectivity’s, a chronic vascular inflammatory disease that affects the skin and the striated muscular system. It is very rare, affects 1 to 4 people per million, with two peaks in incidence at 5-14 years and 45-64 years. Girls are 5 times more affected than boys. It is an acquired condition even though there is sometimes a predisposing genetic background with immunological disorders.

The diagnosis is based on the criteria of Bohan and Peter (1975): the combination of skin signs (edema and erythema), muscle damage (weakness in the roots of the limbs) and the provision of muscle biopsy.

Treatment is based on systemic corticosteroid therapy and immunosuppressants.

The progression is either towards a final cure, worsening of the initial picture in case of resistance to treatment, or by fatal complications during relapses.

The aim of our work

Make this pathology known through our patient, which can lead to confusion with chronic juvenile arthritis and systemic lupus erythematosus.

The observation

M.A a boy, referred to our service at the age of 8 for suspected rheumatic fever.

History of recurrent angina with febrile arthralgia after being put on Extencillin every 21 days.

History:
Arthralgia with stiffening especially in the morning on waking evolving for 6 months, affecting large and small joints bilaterally and symmetrically.

Repeated mouth ulcers a rash on the face.

Lower back, knees and hands.

The clinical picture on admission:

Erythematous lesions on the face, red-purplish on the upper eyelids and the metacarpophalangeal and interphalangeal joints.

The joints are painful when mobilized.

Exploration para clinic:

The EMG objectifying electrical signs in favour of myogenic impairment,

The increase in muscle enzymes (LDH, CPK, aldolase) and muscle testing demonstrating damage to the neck and trunk muscles.

In front of this table, we retained the diagnosis of dermatomyositis.

Management:

Corticosteroid therapy was initiated at a high dose (2 mg / kg / day) by the oral route

Evolution:

It was marked by insufficient clinical improvement, which led us to the combination of corticosteroid therapy with Methotrexate

Conclusion

Dermatomyositis is a rare disease. Its diagnosis is often confused with other connectivity’s, its evolution is unpredictable requiring monitoring and compliance with treatment with heavy side effects.

References

1.HAS- Protocole National de Diagnostic et de Soins (PNDS) “Dermatomyosite de l’enfant et de l’adulte Ce PNDS a été dirigé par Brigitte Bader-Meunier et Olivier Benveniste sous l'égide et avec le partenariat de la Filière des Maladies AutoImmunes et Auto-Inflammatoires Rares (FAI²R) et de la Filière Neuromusculaire (FILNEMUS)”

2. “Actualités de la dermatomyosite juvenile”- Amine Zouagui. Sana Abourazzak -Mounia LakhdarI- drissi Fatima-   Zohra Souilmi –Sana Chaouki-Samir Atmani- Abdelhak Bouharrou- Moustapha Hida. Revue du Rhumatisme- Volume 78, Issue 3, May 2011, Pages 214-219

Volume 1 Issue 5 November 2020

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