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CLINICAL REPORT 539 Clinical Profile of Methotrexate-resistant Juvenile Localised Scleroderma Juliette HARDY 1 , Franck BORALEVI 2 , Stéphanie MALLET 3 , Natalia CABRERA 4 , Alexandre BELOT 4 , Alice PHAN 4 , Sébastien BARBAROT 5 , Audrey DURIEZ-LASEK 6 , Christine CHIAVERINI 7 , Thomas HUBICHE 8 , Emmanuel MAHE 9 , Edouard BEGON 10 , Emmanuelle BOURRAT 11 , Olivia BOCCARA 12 , Hélène AUBERT 5 , Martine GRALL LEROSEY 13 , Catherine DROITCOURT 14 Maryam PIRAM 15 , Juliette MAZEREEUW-HAUTIER 1 and the Research Group of the French Society of Paediatric Dermatology (SDFP in French) Reference Centre of Rare Skin Diseases, Larrey Hospital, Paul Sabatier University, Toulouse, 2 Department of Paediatric Dermatology, Bordeaux, Departments of Dermatology: 3 Timone Hospital, Marseille, 5 Hôtel-Dieu Hospital, Nantes, 6 Saint-Vincent-de-Paul Hospital, Lille, 7 CHU Nice University Hospital, Nice, 8 Fréjus Hospital, Fréjus, 9 Argenteuil Hospital, Argenteuil, 10 CH de Pontoise University Hospital, Pontoise, 13 CHU de Rouen University Hospital, Rouen and 14 CHU Rennes University Hospital, Rennes, 4 Department of Paediatric Nephrology, Rheumatology & Dermatology, Femme-Mère-Enfant Hospital, Lyon, RAISE referee centre, Lyon, Departments of Paediatric Dermatology, 11 Robert-Debré Hospital, Paris and 12 Necker-Enfants Malades Hospital, Paris, and 15 Department of Paediatric Rheumatology, Bicêtre Hospital, AP-HP, University Paris Sud, Le Kremlin-Bicêtre, France 1 Methotrexate has demonstrated its efficiency for the treatment of juvenile localized scleroderma but some patients may be resistant. The aim of our study was to define the profile of such patients. We performed an observational retrospective multicenter study bet- ween 2007 and 2016 and included all children seen in the French Paediatric Dermatology and Rheumato- logy departments with active localized scleroderma treated by methotrexate for a minimum of 4 months. Metho­trexate efficacy was assessed clinically and/or by imaging between the fourth to twelfth months of treatment. A total of 57 patients were included. Metho­ trexate dosage ranged from 7 to 15 mg/m 2 /week. Only 4 patients were resistant. No common features could be identified between these 4 patients. Child- ren with localized scleroderma are rarely resistant to metho ­ trexate and we did not identify a clinical profile for those resistant patients. Key words: localized scleroderma; morphoea; scleroderma; treatment; methotrexate. Accepted Feb 26, 2019; E-published Feb 27, 2019 Acta Derm Venereol 2019; 99: 539–543. Corr: Dr. Juliette Hardy, Reference Centre of Rare Skin Diseases, Larrey Hospital, Paul Sabatier University, FR-31400 Toulouse, France. E-mail: [email protected] L ocalised scleroderma (LS) or morphoea, is a connec- tive tissue disorder of unknown aetiology characteri- sed by fibrosis of the skin and subcutaneous tissues (1). It is a rare disease more commonly affecting children than adults. The annual incidence rate in childhood is estima- ted at 1 to 2.7 per 100,000 individuals (2). Juvenile LS has been classified by Laxer & Zulian into 5 subtypes (3): linear involving the torso, limbs (most commonly) or the head (“en coup de sabre” and Parry–Romberg syndrome); plaque-type; generalised; disabling pansclerotic mor­ phoea; and mixed morphoea. Early lesions commonly present as erythematous patches that evolve into sclerotic plaques. Parry–Romberg syndrome is characterised by hemifacial atrophy involving the subcutis and bone with SIGNIFICANCE Morphea is a rare skin condition due, in part, to an unusual reaction of the immune system. Disease generally affects the outermost layers of the skin which becomes hard and thickened. In severe cases underlying tissue and bones can be affected resulting in functional disabilities. Metho­ trexate in association with systemic corticosteroids are the most frequent drugs prescribed for severe form of the disease. However some patient will not respond to these treatments. The aim of our study was to better define the clinical profile of these resistant patients in order to help clinicians in their therapeutic choice. mobile overlying skin without sclerosis. In contrast to systemic sclerosis, LS is considered a benign disease but can be complicated by joint contractures and limitations, limb length discrepancy and deformities. Neurological (4) (epilepsy, migraine, neuralgia and/or paraesthesia of cranial nerves) and ophthalmological (adnexa abnormali- ties, uveitis, episcleritis) (5) anomalies may be associated with LS localised to the head. LS usually last for several years, sometimes up to 20 years, with long stretches of quiescence interrupted by unpredictable reactivations (6). Disease activity is difficult to evaluate because of a lack of specific biomarkers. Imaging is performed in some centres. Doppler ultrasound may identify areas of increased blood flow related to inflammation (7). Magnetic resonance imaging reveals a thickening of the dermis and infiltration of the subcutaneous fatty tissue with an increased signal intensity (8). Assessment Tool (LoSCAT) and Computerized Skin Score (CSS) have also been proposed for disease activity monitoring as well as imaging techniques such infrared thermography (IRT), laser doppler flowmeter and, more recently, Cone Beam Computed Tomography (CBCT) (9–11). Many topical and systemic therapies have been re- ported in the literature for juvenile LS, with variable efficiency (12). A few randomised controlled studies are available and management of LS depends, in clinical This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3155 Acta Derm Venereol 2019; 99: 539–543