Fibrous Arthropathy Associated With Etienne Merlin, MD, PhD, a Sylvain Breton, MD, b Sylvie Fraitag, MD, PhD, c Jean-Louis Stéphan, MD, PhD, d Morphea:Carine Wouters, MD, PhD, e Christine A Bodemer,New MD, PhD, Cause f Brigitte Bader-Meunier, of DiffuseMD, PhDg 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, aInserm CIC 1405, Centre Hospitalier Universitaire de dermatomyositis, and systemic sclerosis. We report on 2 boys, aged 5 and Clermont-Ferrand, Clermont-Ferrand, France; bRadiologie 8 years, who developed acquired symmetric painless joint contractures Pédiatrique, Hôpital Necker Enfants Malades, Assistance Publique – Hopitaux de Paris, Paris, France; cService preceding the development of superficial plaques of morphea by 7 to 13 d’Anatomie et de Cytologie Pathologiques, Hôpital Necker months. There was no other clinical involvement, biological inflammation, Enfants Malades, Assistance Publique – Hopitaux de Paris, d or autoantibodies. No urinary mucopolysaccharidosis was seen. In both Paris, France; Service d’hématologie immunologie et rhumatologie pédiatrique, Centre Hospitalier Universitaire patients, wrist MRI showed no joint effusion, no bone erosion, and no or de Saint-Etienne, Saint-Priest-en-Jarez, France; eDepartment mild synovial thickening with slight enhancement after gadolinium infusion. of Pediatric Rheumatology, University Hospital Leuven, Leuven, Belgium; fService de Dermatologie, Hôpital Necker One patient underwent a synovial biopsy, which showed dense fibrosis Enfants Malades, Institut IMAGINE, Assistance Publique with a sparse inflammatory infiltrate, similar to the pathologic pattern – Hopitaux de Paris, Paris, France; and gUnité d’Immuno- Hématologie et Rhumatologie Pédiatrique, Hôpital Necker observed in the skin biopsy. With methotrexate and systemic steroids, joint Enfants Malades, Institut IMAGINE, Assistance Publique – contractures slowly improved in the first patient and remained stable in the Hopitaux de Paris, Paris, France second. These 2 cases suggest that fibrous synovitis should be considered Dr Merlin drafted the initial manuscript; Dr Breton in children with acquired diffuse, symmetric, painless contractures and performed imaging studies and reviewed and without elevation of acute-phase reactants, even in the absence of cutaneous revised the manuscript; Dr Fraitag performed histological examination and reviewed and manifestations. Articular MRI with gadolinium and careful cutaneous revised the manuscript; Drs Stéphan and Wouters examination at onset and during follow-up should provide clues for participated in the care of the patients and diagnosing this entity. reviewed and revised the manuscript; Drs Bodemer and Bader-Meunier conceptualized the study and reviewed and revised the manuscript; and all the authors approved the final manuscript as submitted. Etiologies of childhood-onset diffuse joint capsule fibrosis. The skin and joint contractures encompass a subcutis in these patients is described DOI: https:// doi. org/ 10. 1542/ peds. 2016- 1899 “ ” large group of inherited disorders as hard and indurated and feels Accepted for publication Feb 14, 2017 and acquired diseases, in particular, tacked down. At times, involvement Address correspondence to Brigitte Bader- subgroups of patients within of deep connective tissue below the Meunier, MD, PhD, Unité d’Immuno-Hématologie et “ ” connective tissue diseases that skin may affect joint functioning Rhumatologie Pédiatrique, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. demonstrate a dry synovitis, which slightly distal to the area. For example, E-mail: [email protected] is the presence of joint contractures an indurated linear patch of LS of the PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, or stiffness without obvious effusion upper arm may affect the functioning 1098-4275). or warmth of the joint. These include of the elbow, wrist, and fingers if the “ ” Copyright © 2017 by the American Academy of juvenile idiopathic arthritis (JIA), deeper tendons are involved, causing a Pediatrics dermatomyositis, systemic sclerosis, pulley effect. The forearm and hand and localized scleroderma (LS), will otherwise appear normal without To cite: Merlin E, Breton S, Fraitag S, et al. Fibrous typically the deep morphea and linear cutaneous findings in these instances. Arthropathy Associated With Morphea: A New morphea subtypes of LS affecting the Here we describe for the first time the Cause of Diffuse Acquired Joint Contractures. extremity. In LS and systemic sclerosis, cases of 2 patients who presented with Pediatrics. 2017;140(4):e20161899 the dry synovitis is associated with diffuse acquired joint contractures deep subcutis, fascial, tendon, and preceding distant superficial Downloaded from www.aappublications.org/news by guest on September 29, 2021 PEDIATRICS Volume 140, number 4, October 2017:e20161899 CASE REPORT Merlin et al 2017 ROUGH GALLEY PROOF Fibrous Arthropathy Associated With https://doi.org/10.1542/peds.2016-1899 October 2017 Morphea: A New Cause of Diffuse Acquired Joint Contractures 4 140 Pediatrics TABLE 1 Characteristics of the Patients Patient 1 Patient 2 circumscribed morphea, without associated deep morphea. This report Age at onset of contractures/sex 8 y/male 5 y/male Joints involved Mild limitations of PIP, DIP, Moderate limitations of PIP, DIP, emphasizes that fibrosing synovitis wrists, elbows, hips, knees, wrists, hips, knees, ankles, should be considered in children with ankles cervical spine acquired contractures, even in the Time from onset of polyarthritis and 7 mo 13 mo absence of cutaneous manifestations. onset of morphea Type of morphea 3 superficial plaques (pubis, 5 superficial plaques (neck, CLINICAL REPORTS back, leg) axilla, pubis, groin) ESR/CRP Normal Normal Eosinophilia/elevated CPK No/no No/no Autoantibodies ANA, anti-SSA, anti-SSB, anti- ANA and RF: negative Two Caucasian boys, aged 5 and 8 RNP, anti-Scl70: negative years, were referred to a pediatric Wrist MRI with gadolinium infusion Mild synovial thickening with No synovial thickening, slight rheumatologist for the evaluation slight enhancement of enhancement of synovial of diffuse joint contracture that had synovial membrane after membrane after gadolinium gadolinium infusion infusion developed some months earlier. No joint effusion, sparse No joint effusion, no bone Their medical history was negative intraosseous edema abnormalities (Table 1). There was no noteworthy without bone erosion family history or consanguinity. Joint Skin biopsy Dermal fibrosis, mild Dermal fibrosis, sparse limitation was graduated from 1 to 3, lymphocytic infiltrate, inflammatory infiltrate horizontalization of as mild (1), moderate (2), or severe elastic fibers and adnexa P(3).atient 1 rarefaction Synovial biopsy Dense fibrosis with very Not done sparse inflammatory infiltrates An 8-year-old boy had diffuse ANA, antinuclear antibodies; CPK, creatinine phosphokinase; CRP, C reactive protein; DIP, distal interphalangeal joint; painless mild joint limitation of ESR, erythrocyte sedimentation rate; NSAID, nonsteroidal antiinflammatory drug; PIP, proximal interphalangeal joint; RF, motion that had developed 5 months rheumatoid factor; RNP, ribonucleoprotein; SSA and SSB, Sjögren syndrome A and B. earlier, involving the proximal and distal interphalangeal joints, wrists, hips, knees, and ankles. The severity of joint limitations was mild with the patient began treatment2 with <15 minutes. No other clinical no functional impact. Seven months methotrexate (10 mg/m /week) abnormalities were noted. Wrist after the onset of joint involvement, combined with corticosteroids x-rays, ophthalmologic examination 3 hyperpigmented superficial (1 mg/kg/day prednisone for 1 (including slit-lamp examination), cutaneous plaques suggestive of month then tapered to 5 mg/day) and echocardiography were normal, morphea had appeared on the pubis, for 6 months. In the absence of along with acute phase reactants. back, and leg. Laboratory tests found any articular improvement, a wrist Wrist MRI showed no joint effusion, normal complete blood cell count synovial biopsy was performed, no bone abnormalities, no synovial with differential and C-reactive which showed dense fibrosis with thickening, no subcutis/fascial protein level. Rheumatoid factor and a sparse inflammatory infiltrate edema, but mild enhancement of the antinuclear antibody were negative (Fig 2). Methotrexate and steroids carpal synovium after gadolinium (single-strand DNA and histone were stopped after 6 months, and infusion. Laboratory results are antibodies were not tested). Joint physiotherapy was pursued. Morphea given in Table 1. Nonsteroidal x-rays (hips, knees, ankles, wrists, remitted within the next 4 months, antiinflammatory drugs provided a leaving residual hyperpigmentation and spine), echocardiography, eye mild subjective improvement,2 and examination, and pulmonary function without induration. One year after methotrexate (15 mg/m /week) tests were normal. Ultrasonography the treatment discontinuation, the combined with steroids (10 mg/day and MRI showed no joint effusion or patient was well with stable mild prednisolone 6 weeks, then 7.5 mg/ bone erosions, but a mild synovial Pjointatient limitations. 2 day 6 weeks, then 5 mg/day 8 weeks, thickening slightly enhanced then 2.5