Fibrous Arthropathy Associated With Morphea: A New Cause of Diffuse Acquired Joint Contractures

Abstract

Etiologies for childhood-onset diffuse joint contractures encompass a large group of inherited disorders and acquired diseases, in particular a subtype of juvenile idiopathic arthritis called "dry polyarthritis," dermatomyositis, and systemic sclerosis. We report on 2 boys, aged 5 and 8 years, who developed acquired symmetric painless joint contractures preceding the development of superficial plaques of morphea by 7 to 13 months. There was no other clinical involvement, biological inflammation, or autoantibodies. No urinary mucopolysaccharidosis was seen. In both patients, wrist MRI showed no joint effusion, no bone erosion, and no or mild synovial thickening with slight enhancement after gadolinium infusion. One patient underwent a synovial biopsy, which showed dense fibrosis with a sparse inflammatory infiltrate, similar to the pathologic pattern observed in the skin biopsy. With methotrexate and systemic steroids, joint contractures slowly improved in the first patient and remained stable in the second. These 2 cases suggest that fibrous synovitis should be considered in children with acquired diffuse, symmetric, painless contractures and without elevation of acute-phase reactants, even in the absence of cutaneous manifestations. Articular MRI with gadolinium and careful cutaneous examination at onset and during follow-up should provide clues for diagnosing this entity.

Dry synovitis, a rare entity distinct from juvenile idiopathic arthritis

“…Interestingly, Merlin et al recently published the case of two boys who developed symmetric painless joint contractures preceding by 6 and 12 months the development of superficial plaques of morphea [20]. MRI imaging was similar to what is seen in our DS patients showing absent to moderate thickening of the synovium with mild enhancement after gadolinium infusion.…”

Synovitis and tenosynovitis of the hands and wrists in patients with SSc: The need for a consensus definition of sclerosing tenosynovitis

“…Fibrotic/sclerosing tenosynovitis is more frequent in patients with the diffuse cutaneous subset (dcSSc), and could participate to the phenomenon of tendon friction rubs in this population [2,5]. Although mostly considered specific to SSc, such sclerosing/fibrotic tenosynovitis has been recently described in linear morphea in children [6,7]. This existence of fibrotic tenosynovitis in pediatric cases challenges the hypothesis that osteophytosis could participate in the pathogenesis of fibrotic tenosynovitis, considering the low prevalence of osteophytes in this younger population.…”

Treatment of juvenile localized scleroderma: current recommendations, response factors, and potential alternative treatments

“…Specific extracutaneous problems, such as arthritis and arthropathy, have also be found less responsive to MTX treatment than skin disease. Among four studies reporting on 25 jLS patients with arthritis and fibrous arthropathy, over 75% received additional medications, with joint disease still refractory in 32% [17 ▪ ,18–20]. In another study, muscle involvement was present in 39% and bone growth impairment in 26% of patients despite more than 80% of the patients having received or receiving MTX and corticosteroid treatment [3 ▪ ].…”