Parry Romberg disease and En Coupe de Sabre Scleroderma are frequently associated disorders that affect the face and can cause severe aesthetic and functional impairment. Systemic immunosuppression is the gold standard of first-line treatment in the pediatric rheumatology standpoint although it is often delayed in the pediatric dermatology clinics and more often used in cases of refractory neurological impairment. We report on a case with dental root resorption and severe periodontal bone inflammation detected on magnetic resonance imaging, which was successfully treated with the anti-IL-6 agent tocilizumab.
GlaserDHSchuttCVonVilleHM, et al. Linear scleroderma of the head – updates in management of parry Romberg syndrome and en coup de sabre: a rapid scoping review across subspecialties. Eur J Rheumatol2020; 17(7): 48–57.
2.
LiSCTorokKSPopeE, et al. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res2012; 64(8): 1175–1185.
3.
AndersonLETreatJRLichtDJ, et al. Remission of seizures with immunosuppressive therapy in Parry-Romberg syndrome and en coup de sabre linear scleroderma: case report and brief review of the literature. Pediatr Dermatol2018; 35(6): e363–e365.
4.
MagroCMHaltehPOlsonLC, et al. Linear scleroderma ‘en coup de sabre’ with extensive brain involvement – clinicopathologic correlations and response to anti-Interleukin-6 therapy. Orphanet J Rare Dis2019; 14(1): 1–7.
5.
KhamaganovaI.Progressive hemifacial atrophy and linear scleroderma en coup de sabre: a spectrum of the same disease. Front Med2017; 4: 258–258.
6.
ChiuYEVoraSKwon MaheshwariM.A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol2012; 29(6): 738–748.
7.
KorkmazCAdapinarBUysalS.Beneficial effect of immunosuppressive drugs on Parry-Romberg syndrome: a case report and review of the literature. South Med J2005; 98(9): 940–942.
8.
TollefsonMMChiuYEBrandling-BennettHA, et al. Discordance of pediatric morphea treatment by pediatric dermatologists. Pediatr Dermatol2018; 35(1): 47–54.
9.
MisraDPAhmedSAgarwalV.Is biological therapy in systemic sclerosis the answer. Rheumatol Int2020; 40(5): 679–694.
10.
KhannaDDentonCPLinCJF, et al. Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis2018; 77(2): 212–220.
11.
KhannaDLinCJFFurstDE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med2020; 8(10): 963–974.
12.
DistlerODistlerJHW. Tocilizumab for systemic sclerosis: implications for future trials. Lancet2016; 387: 2580–2581.
13.
FoeldvariIAntonJFriswellM, et al. Tocilizumab is a promising treatment option for therapy resistant juvenile localized scleroderma patients. J Scleroderma Relat Disord2017; 2(3): 203–207.
14.
Shahidi-DadrasMAbdollahimajdFJahangardR, et al. Magnetic resonance imaging evaluation in patients with linear morphea treated with methotrexate and high-dose corticosteroid. Dermatol Res Pract2018; 839: 1218–1216.
15.
SchanzSFierlbeckGUlmerA, et al. Localized scleroderma: MR findings and clinical features. Radiology2011; 260(3): 817–824.
16.
Demirturk KocasaracHGehaHGaalaasLR, et al. MRI for dental applications. Dent Clin North Am2018; 62(3): 467–480.
17.
ConstantinTFoeldvariIPainCE, et al. Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr2018; 177(7): 961–977.
18.
ZulianFCulpoRSperottoF, et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis2019; 78(8): 1019–1024.
19.
O’FlynnSKinironsM.Parry-Romberg syndrome: a report of the dental findings in a child followed up for 9 years. Int J Paediatr Dent2006; 16(4): 297–301.
