[Infantile dermatomyositis. Clinical aspects and prospective treatments].
Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.
Cordone
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G
I Clinica Pediatrica G. Gaslini, Università di Genova.
Buoncompagni
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A
Ciccone
O
O
Minetti
C
C
Fasce
L
L
Scribanis
R
R
De Langlade
E
E
D000208:Acute Disease; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D003882:Dermatomyositis / Q000175:diagnosis / Q000188:drug therapy; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008297:Male
D000305:Adrenal Cortex Hormones; D016572:Cyclosporine; D003520:Cyclophosphamide
Dermatomiosite infantile. Aspetti clinici e prospettive terapeutiche.