Dermatomyositis and polymyositis in childhood. 1994

H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
Department of Pediatrics, Chang Gung Memorial Hospital Taipei, Taiwan, R.O.C.

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.

UI MeSH Term Description Entries
D008297 Male Males
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003882 Dermatomyositis A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012737 Sex Factors Maleness or femaleness as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or effect of a circumstance. It is used with human or animal concepts but should be differentiated from SEX CHARACTERISTICS, anatomical or physiological manifestations of sex, and from SEX DISTRIBUTION, the number of males and females in given circumstances. Factor, Sex,Factors, Sex,Sex Factor
D017285 Polymyositis Diseases characterized by inflammation involving multiple muscles. This may occur as an acute or chronic condition associated with medication toxicity (DRUG TOXICITY); CONNECTIVE TISSUE DISEASES; infections; malignant NEOPLASMS; and other disorders. The term polymyositis is frequently used to refer to a specific clinical entity characterized by subacute or slowly progressing symmetrical weakness primarily affecting the proximal limb and trunk muscles. The illness may occur at any age, but is most frequent in the fourth to sixth decade of life. Weakness of pharyngeal and laryngeal muscles, interstitial lung disease, and inflammation of the myocardium may also occur. Muscle biopsy reveals widespread destruction of segments of muscle fibers and an inflammatory cellular response. (Adams et al., Principles of Neurology, 6th ed, pp1404-9) Myositis, Multiple,Polymyositis Ossificans,Polymyositis, Idiopathic,Idiopathic Polymyositides,Idiopathic Polymyositis,Multiple Myositis,Myositides, Multiple,Ossificans, Polymyositis,Polymyositides,Polymyositides, Idiopathic

Related Publications

H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
January 1988, Clinics in dermatology,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
January 1990, Brain & development,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
October 1988, Anales espanoles de pediatria,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
April 1979, American journal of diseases of children (1960),
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
September 2003, Current treatment options in neurology,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
January 1975, Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
February 1962, Bulletin on the rheumatic diseases,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
July 1960, The Medical clinics of North America,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
January 1993, Nihon rinsho. Japanese journal of clinical medicine,
H T Chung, and J L Huang, and H S Wang, and P C Hung, and M L Chou
May 1971, Rocky Mountain medical journal,
Copied contents to your clipboard!