Juvenile Dermatomyositis: A Clinical Overview

Pachman, Lauren M

doi:10.1542/pir.12-4-117

Cited by 23 publications

(15 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The histological muscle features of the disease (inflammation, necrosis and regeneration of muscle fibers and perifascicular atrophy) are characteristic 8 In the present work, we describe the histological and histochemical findings of skeletal muscle biopsy in 10 patients withjuvenile dermatomyositis. We try to correlate these findings with the clinicaI response to the treatment and the clinicaI recurrence.…”

Section: Andress For Correspondence: Edenilson Eduardo Calore Institumentioning

confidence: 92%

Muscle pathology in juvenile dermatomyositis

1997

View full text Add to dashboard Cite

Objective: To study muscle biopsies, using histochemistry, on ten children with infantile dermatomyositis. Design: Series of ten patients (of whom eight patients had received treatment and two had not) were submitted to muscle biopsy in order to diagnose possible inflammatory myopathy or to detect rec~rrences. Place of development of the study: Public Health Service of São Paulo State. Participants: children with clinicai features of inflammatory myopathy. Intervention: biopsies were performed on the vastus lateralis using local anesthetic. Histochemistry was performed according to standardized methods. Results: Architectural changes of the muscle fibers, necrosis of variable intensity and accentuated evidence of regeneration were observed in patients who had not received treatment (2 cases) and in one case where muscular weakness persisted in spite of corticosteroid therapy. Necrosis and regeneration were minimal or absent in cases treated for one year or more (4 cases). In 3 cases with clinicai and laboratorial recurrences, muscle necrosis and architectural changes were detected. Conclusions: It was concluded that muscle biopsy could aid in diagnosing infantile dermatomyositis as well as in detecting recurrences even in cases without clinicai activity of the disease.

show abstract

Section: Andress For Correspondence: Edenilson Eduardo Calore Institumentioning

confidence: 92%

Muscle pathology in juvenile dermatomyositis

1997

View full text Add to dashboard Cite

show abstract

“…The skin changes seen in children with DM are similar, if not identical, to those seen in the adult form of the disease, except that calcinosis is much more common in children (3,4). Periorbital, violaceous erythema with or without edema, known as the heliotrope rash, is pathognomonic of DM.…”

mentioning

confidence: 84%

Photodermatitis in a 6‐Year‐Old Child

Callen

1993

Arthritis & Rheumatism

View full text Add to dashboard Cite

A 6-year-old Caucasian boy was referred for evaluation of a facial rash that had begun 2 months earlier. He had been initially examined by his pediatrician, who believed the patient had erythema infectiosum and did not prescribe any treatment. After 2 weeks the patient's rash was unchanged or slightly worse, and he was referred to a local dermatologist whose initial impression was that the patient had mild seborrhea with features of psoriasis on the elbows. Mometasone furoate ointment and a tar-based shampoo were prescribed for topical application. Within an additional 2 weeks it was evident that the facial "butterfly" rash was in a photosensitive distribution, and lesions were now also present on the dorsum of the hands. The diagnoses considered were photosensitivity dermatitis and systemic lupus erythematosus (SLE). Antinuclear antibody (ANA) testing was ordered, and the patient was prescribed a broadspectrum sunscreen and a tapering course of oral methylprednisolone over a 21-day period. ANA was found to be positive (using HEp-2 cells as substrate) at a titer of >1:640, in a speckled pattern. Other autoantibodies, specifically anti-native DNA, anti-Sm, and anti-Ro/SS-A, were negative. He was then referred to this institution for further evaluation and treatment.Physical examination revealed an erythematous rash across the malar surface (Figure 1). There was a slight edema and erythema of the upper eyelids. The dorsal areas of the hands had an erythematous rash

show abstract

“…Hydroxychloroquine has been successfully used to treat the cutaneous manifestations of DM in both adults and children, [32][33][34] and is the preferred agent for use in children due to its favorable risk-benefit ratio. Mepacrine (quinacrine) and chloroquine are also effective but are less commonly utilized because of a greater incidence of adverse effects.…”

Section: Antimalarialsmentioning

confidence: 99%

“…[6,[13][14][15]34,38] In some cases, methotrexate may be used initially to avoid corticosteroid adverse effects. The most commonly employed treatment protocols involve the administration of systemic corticosteroids (either oral prednisone or intravenous methylprednisolone) as initial monotherapy; patients who do not show an adequate response to this treatment may be additionally prescribed a nonsteroidal immunosuppressant medication, most commonly methotrexate.…”

Section: Systemic Corticosteroidsmentioning

confidence: 99%

Juvenile-Onset Clinically Amyopathic Dermatomyositis

Walling¹,

Gerami

Sontheimer

2010

Pediatric Drugs

View full text Add to dashboard Cite

Juvenile-onset amyopathic dermatomyositis is an uncommon variant of juvenile-onset dermatomyositis (JDM), characterized by the hallmark cutaneous features of dermatomyositis for at least 6 months without clinical or laboratory evidence of muscle disease. Cutaneous calcinosis, vasculopathy, and interstitial lung disease frequently complicate the course of classic JDM (typical JDM with myositis) but are infrequent in amyopathic JDM. Recent literature suggests that approximately 75% of amyopathic JDM patients will remain free from muscle disease after years of follow-up, while approximately 25% of patients will evolve to having classic JDM. No clinical, laboratory, or ancillary parameters have been found to be predictive for this transition to muscle disease. Treatment of the cutaneous disease of amyopathic JDM centers on photoprotection and topical therapies directed against inflammation. Oral antimalarials are effective for cutaneous disease not adequately controlled with topical care. Systemic corticosteroids, while central to the treatment of classic JDM, are controversial in the treatment of amyopathic JDM. Randomized controlled trials are not available to guide the management of this disease. Proponents for early aggressive systemic corticosteroid therapy for amyopathic JDM advocate that this intervention may decrease the likelihood of progression to classic JDM, and/or prevent disease-specific complications of JDM such as calcinosis. Opponents of early intervention with systemic corticosteroids favor expectant management directed toward controlling skin disease, citing the predictable adverse effects of systemic corticosteroids in the face of uncertain benefit. Other therapeutic options for severe and recalcitrant cutaneous disease, including methotrexate, intravenous immunoglobulin, and rituximab, are reviewed, as are treatment options for calcinosis cutis. In weighing the available evidence, the authors conclude that early aggressive treatment of amyopathic JDM with systemic immunosuppressant agents should be avoided in most cases as the risk of these medications will outweigh the measurable benefit. The reported literature suggests a good prognosis for amyopathic JDM. Ongoing clinical follow-up is recommended in all cases to allow early detection of subtle signs of muscle disease.

show abstract

Juvenile Dermatomyositis: A Clinical Overview

Cited by 23 publications

References 0 publications

Muscle pathology in juvenile dermatomyositis

Muscle pathology in juvenile dermatomyositis

Photodermatitis in a 6‐Year‐Old Child

Juvenile-Onset Clinically Amyopathic Dermatomyositis

Contact Info

Product

Resources

About