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Peculiarities of dermatomyositis (DM) in early age
T V Ryabova1, M V Velikoretskaya, L I Kotova
1First Moscow Medical Institute, USSR.
Insights
This study identifies key risk factors and early symptoms of childhood dermatomyositis. Findings highlight the importance of family history and specific clinical signs in diagnosing this rare pediatric rheumatic disease.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Genetics
Background:
- Childhood dermatomyositis is a rare autoimmune disease.
- Understanding its onset and risk factors is crucial for early diagnosis and management.
Purpose of the Study:
- To investigate the peculiarities of disease onset and clinical risk factors in children with dermatomyositis.
- To identify potential genetic predispositions and early clinical markers.
Main Methods:
- Follow-up study of 70 children diagnosed with dermatomyositis (ages 14 months to 8 years).
- Analysis of intercurrent illnesses, allergies, preceding factors (infections, inoculations), and family history (genealogical investigations).
- Assessment of dermatological indices and developmental anomalies.
Main Results:
- Most children had normal early development; frequent preceding factors included respiratory infections and inoculations.
- Genealogical studies revealed a high incidence of rheumatological conditions in affected families.
- Acute or subacute onset occurred in two-thirds of cases, with characteristic skin manifestations like facial erythema and livedo reticularis.
Conclusions:
- Early identification of specific clinical signs and a positive family history are vital for diagnosing childhood dermatomyositis.
- Further research into genetic and environmental factors may improve risk stratification and prevention strategies.
Abstract:
The peculiarities of beginning of disease, clinical factors of risk in 70 children (aged under 8) with dermatomyositis are discussed. We have followed up 70 children (40 girls and 30 boys) with dermatomyositis at the age of 14 months to the age of 8 years. Most of the children were born in normal time, they have normal life functions during the first year of life. The most frequent intercurrent diseases were respiratory infections, angina; the chicken-pox was frequent too. 12 children had food allergy and 20 children-drug allergy. The most frequent factors preceded dermatomyositis were respiratory diseases and inoculations. Assembling of genealogical anamnesis was made according to "family portrait". 14 families of children with dermatomyositis (369 relations in I-IV degree of relationship) were examined. Genealogical investigations revealed the high frequency of rheumatological pathology in proband's families. Small anomaly of development among probands (M +/- m = 12.5 +/- 0.6) exceeded these quantity comparing to the control group (n = 60). Dermatollphyics (98 indices on each child) contained a number of peculiarities for forming the group of risk. Onset of disease was acute or subacute in 2/3 of children. Primary chronic onset and the progress of the disease took place in 10 cases. An acute onset of dermatomyositis was characterized by fever, myaglia, arthralgia, bright skin symptoms: widespread purple violet face erythema, a "butterfly wing" or "paraorbital glasses", palmal erythema and widespread vascular manifestations on face, chest, back and limbs in the form of net--"livedo reticularis".