Zulian F et al., 2019. Consensus-based recommendations for the management of juvenile localised scleroderma.Ann Rheum Dis 78: 1019–1024.
Zulian F, Vallongo C, de Oliveira SK, Punaro MG, Ros J, Mazur-Zielinska H, Galea P, Da Dalt L, Eichenfield LF, 2006. Congenital localized scleroderma. J Pediatr 149: 248–251.
Makhakhe L, Dlova N, Smith A, du Preez M, 2022. En coup de sabre morphea: An uncommon condition in Africa. Dermatol Rep 15: 9537.
Fett N, Werth VP, 2011. Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 64: 217–230.
Erzer JN, Jaeger VK, Tikly M, Walker UA, 2020. Systemic sclerosis in sub-Saharan Africa: A systematic review. Pan Africa Med J 37: 176.
Abdelnour JGW, Abdelnour YGW, Kerollos RAB, Mahmoud ZIT, 2019. Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan—A rare entity from East Africa: A case report. J Med Case Rep 13: 138.
Hasan S, Aqil M, Panigrahi R, 2022. HIV-associated systemic sclerosis: Literature review and a rare case report. Int J Environ Res Public Health 19: 10066.
Okong’o LO, Webb K, Scott C, 2015. HIV-associated juvenile systemic sclerosis: A case report. Semin Arthritis Rheum 44: 411–416.
Calabrese LH, Kirchner E, Shrestha R, 2005. Rheumatic complications of human immunodeficiency virus infection in the era of highly active antiretroviral therapy: Emergence of a new syndrome of immune reconstitution and changing patterns of disease.Semin Arthritis Rheum 35: 166–174.
Franco JP, Serra MS, Lima RB, D’Acri AM, Martins CJ, 2016. Scleroderma en coup de sabre treated with polymethylmethacrylate—Case report. An Bras Dermatol 91: 209–211.
Saracino AM, Denton CP, Orteu CH, 2017. The molecular pathogenesis of morphoea: From genetics to future treatment targets. Br J Dermatol 177: 34–46.
Piram M, McCuaig CC, Saint-Cyr C, Marcoux D, Hatami A, Haddad E, Powell J, 2013. Short- and long-term outcome of linear morphoea in children. Br J Dermatol 169: 1265–1271.
Zulian F et al.; Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES), 2005. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52: 2873–2881.
Appenzeller S, Montenegro MA, Dertkigil SS, Sampaio-Barros PD, Marques-Neto JF, Samara AM, Andermann F, Cendes F, 2004. Neuroimaging findings in scleroderma en coup de sabre. Neurology 62: 1585–1589.
Niklander S, Marín C, Martínez R, Esguep A, 2017. Morphea “en coup de sabre”: An unusual oral presentation. J Clin Exp Dent 9: e315–e318.
Graham PM, Gupta N, Altman DA, 2019. En coup de sabre. Cutis 103: 34–36.
Zulian F et al.; Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES), 2006. Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 45: 614–620.
Ulc E, Rudnicka L, Waśkiel-Burnat A, Warszawik-Hendzel O, Niemczyk A, Olszewska M, 2021. Therapeutic and reconstructive management options in scleroderma (morphea) en coup de sabre in children and adults. A systematic literature review. J Clin Med 10: 4517.
Joly P, Bamberger N, Crickx B, Belaich S, 1994. Treatment of severe forms of localized scleroderma with oral corticosteroids: Follow-up study on 17 patients. Arch Dermatol 130: 663–664.
Adizie T, Moots RJ, Hodkinson B, French N, Adebajo AO, 2016. Inflammatory arthritis in HIV positive patients: A practical guide. BMC Infect Dis 16: 100.
Mertens JS, Seyger MM, Kievit W, Hoppenreijs EP, Jansen TL, van de Kerkhof PC, Radstake TR, de Jong EM, 2015. Disease recurrence in localized scleroderma: A retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br J Dermatol 172: 722–728.
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Coup de Sabre is a subset of localized linear scleroderma disease influenced by autoimmune, vascular, and genetic factors. The disease often follows an active, relapsing, and remitting course causing the development of permanent sequelae, such as tissue atrophy, pigment alterations, and bony deformities. Treatment strategies have ranged from surgical to injectable and medical management approaches with varying success. Although more common in pediatric patients, Coup de Sabre remains rare, with limited literature available on disease course and treatment outcomes, particularly in the sub-Saharan African population. Furthermore, the existence of autoimmune conditions occurring simultaneously with HIV infection presents an interesting discussion of the interplay between antiretroviral medication, immune dysregulation, and autoimmunity. This case report describes an 11-year-old HIV-positive male in Lilongwe, Malawi with mixed subtype of localized scleroderma manifesting most prominently as Coup de Sabre that remained undiagnosed for 7 years.
Current contact information: Katherine Naeger, Kia Quinlan, and Marietta DeGuzman, Texas Children’s Hospital, Houston, TX, E-mails: katherine.naeger@bcm.edu, kia.quinlan@bcm.edu, and mmd@bcm.edu. Kelvin Jobo, Brigid E. O’Brien, and Jacob L. Todd, Baylor College of Medicine Children’s Foundation Malawi, Lilongwe, Malawi, E-mails: kjobo@baylor-malawi.org, jacob.todd@bcm.edu, and brigid.o’brien@bcm.edu.
Zulian F et al., 2019. Consensus-based recommendations for the management of juvenile localised scleroderma.Ann Rheum Dis 78: 1019–1024.
Zulian F, Vallongo C, de Oliveira SK, Punaro MG, Ros J, Mazur-Zielinska H, Galea P, Da Dalt L, Eichenfield LF, 2006. Congenital localized scleroderma. J Pediatr 149: 248–251.
Makhakhe L, Dlova N, Smith A, du Preez M, 2022. En coup de sabre morphea: An uncommon condition in Africa. Dermatol Rep 15: 9537.
Fett N, Werth VP, 2011. Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 64: 217–230.
Erzer JN, Jaeger VK, Tikly M, Walker UA, 2020. Systemic sclerosis in sub-Saharan Africa: A systematic review. Pan Africa Med J 37: 176.
Abdelnour JGW, Abdelnour YGW, Kerollos RAB, Mahmoud ZIT, 2019. Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan—A rare entity from East Africa: A case report. J Med Case Rep 13: 138.
Hasan S, Aqil M, Panigrahi R, 2022. HIV-associated systemic sclerosis: Literature review and a rare case report. Int J Environ Res Public Health 19: 10066.
Okong’o LO, Webb K, Scott C, 2015. HIV-associated juvenile systemic sclerosis: A case report. Semin Arthritis Rheum 44: 411–416.
Calabrese LH, Kirchner E, Shrestha R, 2005. Rheumatic complications of human immunodeficiency virus infection in the era of highly active antiretroviral therapy: Emergence of a new syndrome of immune reconstitution and changing patterns of disease.Semin Arthritis Rheum 35: 166–174.
Franco JP, Serra MS, Lima RB, D’Acri AM, Martins CJ, 2016. Scleroderma en coup de sabre treated with polymethylmethacrylate—Case report. An Bras Dermatol 91: 209–211.
Saracino AM, Denton CP, Orteu CH, 2017. The molecular pathogenesis of morphoea: From genetics to future treatment targets. Br J Dermatol 177: 34–46.
Piram M, McCuaig CC, Saint-Cyr C, Marcoux D, Hatami A, Haddad E, Powell J, 2013. Short- and long-term outcome of linear morphoea in children. Br J Dermatol 169: 1265–1271.
Zulian F et al.; Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES), 2005. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52: 2873–2881.
Appenzeller S, Montenegro MA, Dertkigil SS, Sampaio-Barros PD, Marques-Neto JF, Samara AM, Andermann F, Cendes F, 2004. Neuroimaging findings in scleroderma en coup de sabre. Neurology 62: 1585–1589.
Niklander S, Marín C, Martínez R, Esguep A, 2017. Morphea “en coup de sabre”: An unusual oral presentation. J Clin Exp Dent 9: e315–e318.
Graham PM, Gupta N, Altman DA, 2019. En coup de sabre. Cutis 103: 34–36.
Zulian F et al.; Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES), 2006. Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 45: 614–620.
Ulc E, Rudnicka L, Waśkiel-Burnat A, Warszawik-Hendzel O, Niemczyk A, Olszewska M, 2021. Therapeutic and reconstructive management options in scleroderma (morphea) en coup de sabre in children and adults. A systematic literature review. J Clin Med 10: 4517.
Joly P, Bamberger N, Crickx B, Belaich S, 1994. Treatment of severe forms of localized scleroderma with oral corticosteroids: Follow-up study on 17 patients. Arch Dermatol 130: 663–664.
Adizie T, Moots RJ, Hodkinson B, French N, Adebajo AO, 2016. Inflammatory arthritis in HIV positive patients: A practical guide. BMC Infect Dis 16: 100.
Mertens JS, Seyger MM, Kievit W, Hoppenreijs EP, Jansen TL, van de Kerkhof PC, Radstake TR, de Jong EM, 2015. Disease recurrence in localized scleroderma: A retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br J Dermatol 172: 722–728.
Past two years | Past Year | Past 30 Days | |
---|---|---|---|
Abstract Views | 1273 | 1273 | 899 |
Full Text Views | 23 | 23 | 10 |
PDF Downloads | 26 | 26 | 13 |