Childhood Scleromyositis: An Overlap Syndrome Associated with PM‐Scl Antibody

Abstract

Fourteen patients with childhood scleromyositis followed from 1 to more than 10 years experienced concomitant sclerodermoid and dermatomyositis features, variably expressed at one time or another during the course of the disease. The most characteristic features were myalgia-myositis, arthralgia-arthritis, puffy, atrophic, sclerotic fingers, and Raynaud's phenomenon. This overlap syndrome was the most frequent sclerodermoid condition in children, differing from both systemic scleroderma and dermatomyositis. The course of the disease was protracted and rather benign, and PM-Scl antibody was an important diagnostic and prognostic marker. We present criteria for diagnosis of scleromyositis and its differentiation from systemic scleroderma, dermatomyositis, and Sharp overlap syndrome.

Scleromyositis: a scleroderma/polymyositis overlap syndrome

“…Jablonska and M. Blaszcyk abnormalities in almost 22% of patients [2]. However, calcinosis was more frequent and more extensive (18% vs. 9.8% in adults).…”

Systemische Sklerose – Diagnose und Klassifikation

“…3 Nekrosen an den Kuppen von Fingern oder Zehen oder grübchenförmige Narben oder akraler digitaler Substanzverlust. 4 Mattenförmige Erweiterungen kleiner Hautgefäße.…”

“…Die Variabilität der Krankheitsausprägung (Symptom, Ausdehnung, Organbefall) erschwert die Bildung klar definierter Gruppen. Die Fokussierung auf eine definierte Mindestausprä-gung nach Schadenskriterien schließt weniger stark ausgeprägte, in der Regel frü-he Fälle als nichtklassifizierbare (undifferenzierte; [17]) Erkrankungen aus oder ordnen mehrfach klassifizierbare Fälle (Mischformen, Overlap-Syndrom; [3,30]) klinischen Sonderformen zu, die mehr als 1/3 der Patienten mit klinischen Symptomen der SSc umfassen.…”

Four Cases of Anti-PM/Scl Antibody-positive Juvenile Overlap Syndrome with Features of Myositis and Systemic Sclerosis

“…Relatively few cases of childhood SSc/myositis overlap have been reported 1,2,3,4,5,6,7 . In our case series, 4 patients diagnosed with juvenile overlap syndrome of myositis with SSc are described ( Table 1).…”

“…Overlap SSc/myositis has a relatively good prognosis 2,6 because of the typically mild myositis and good response to corticosteroids 9 . In our 3 patients with longer followup (an average of nearly 13 yrs), symptom control and eventually clinical remission were achieved (with normal muscle enzyme levels, normal muscle strength, and resolution of the JDM-associated rash), supporting a relatively favorable outcome.…”