Limited Cutaneous Scleroderma

Putu Gde Hari Wangsa; Prima Sanjiwani Saraswati Sudarsa; Made Hermina Laksmi

doi:10.15562/ism.v12i3.1181

Limited Cutaneous Scleroderma

Putu Gde Hari Wangsa ,

Prima Sanjiwani Saraswati Sudarsa ,

Made Hermina Laksmi ,

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DOI: https://doi.org/10.15562/ism.v12i3.1181 |
Published: 2021-12-30

Abstract

Introduction: Scleroderma is a rare autoimmune disease of connective tissue characterized by extensive fibrosis, inflammation, and vasculopathy. It classified as limited cutaneous and diffuse based on the degree of skin involvement. The management is still a challenge since it has a high morbidity and mortality rate. This case report aims to understand scleroderma and provide appropriate treatment that can improve the patient's prognosis.

Case report: A 55-year-old female came with complaints of stiff skin on the forearms, lower legs and face since approximately eight months ago, initially on the right and left forearms, then to face, right and left lower legs. On physical examination, there was sclerosis of the skin on the face, forearms and lower legs, and salt and pepper appearance with Rodnan score were 26. The result from histopathological examination were sceleroderma. The patient was diagnosed with limited cutaneous scleroderma. The management consisted of methotrexate, folic acid, sulpha ferrous, vitamins B1, B6, B12 intraorally and 10% urea cream topically. She also was informed to practice moving the hands and fingers slowly.

Conclusion: The diagnosis of limited cutaneous scleroderma is based on history, physical examination and investigations. It is still a life-threatening disease, however multidisciplinary management with early detection and treatment of complications can improve the prognosis.

References

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Anonim. Register Poliklinik Alergi Imunologi Rumah Sakit Umum Pusat Sanglah Denpasar Januari 2019 - Desember 2020. 2020.
Herrick AL. Systemic sclerosis : clinical features and management Key points. Medicine (Baltimore). 2017;1–9.
Hellmann D. Arthritis & Musculoskeletal Disorders. In: Tierney M, McPhee S, Papadakis M, editors. Current Medical Diagnosis & Treatment 5th Ed. New York: Lange Medical Book; 2006. p. 856–70.
Weedon D. Disorders of Collagen. In: Weedon’s Skin Pathology 3rd ed. Philadelphia: Elsevier; 2010. p. 307–10.
Asano Y. Systemic sclerosis. J Dermatol. 2017;2017(October):1–11.
Hinchcliff M, Mahoney J. Towards a new classification of systemic sclerosis. Nat Rev Rheumatol. 2019;(15):456–7.
Pope JE, Johnson SR. New Classification Criteria for Systemic Sclerosis (Scleroderma). Rheum Dis Clin North Am. 2015;41(3):383–98.
Sureshan D, Riyaz N, Thumbayil L. Cross-sectional study on clinical features and histopathology of systemic sclerosis. J Ski Sex Transm Dis. 2019;1(2):77–83.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatology Venereol. 2017;31(9):1401–24.
Sobolewski P, Ma?li?ska M, Wieczorek M, ?agun Z, Malewska A, Roszkiewicz M, et al. Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221–33.
Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;6736(17):1–15.
Showalter K, Gordon JK. Skin Histology in Systemic Sclerosis: a Relevant Clinical Biomarker. Curr Rheumatol Rep. 2021;23(3):1–8.
Ravi D, Prabhu S. Management of Systemic Sclerosis: A Dermatologist’s Approach. Clin Dermatology Rev. 2019;3:34–40.
Hachulla E, Agard C, Allanore Y, Avouac J, Bader-Meunier B, Belot A, et al. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis. 2021;16(Suppl 2):1–61.
Fernández-Codina A, Walker KM, Pope JE. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheumatol. 2018;70(11):1820–8.

[1] Moinzadeh P. Scleroderma. In: Goldsmith L, Katz S, Gilchrest B, Paller A, Leffel D, Wolff K, editors. Fitzpatrick’s Dermatology in General Medicine 9th ed. New York: McGraw-Hill Education; 2019. p. 1942–56.

[2] Anonim. Register Poliklinik Alergi Imunologi Rumah Sakit Umum Pusat Sanglah Denpasar Januari 2019 - Desember 2020. 2020.

[3] Herrick AL. Systemic sclerosis : clinical features and management Key points. Medicine (Baltimore). 2017;1–9.

[4] Hellmann D. Arthritis & Musculoskeletal Disorders. In: Tierney M, McPhee S, Papadakis M, editors. Current Medical Diagnosis & Treatment 5th Ed. New York: Lange Medical Book; 2006. p. 856–70.

[5] Weedon D. Disorders of Collagen. In: Weedon’s Skin Pathology 3rd ed. Philadelphia: Elsevier; 2010. p. 307–10.

[6] Asano Y. Systemic sclerosis. J Dermatol. 2017;2017(October):1–11.

[7] Hinchcliff M, Mahoney J. Towards a new classification of systemic sclerosis. Nat Rev Rheumatol. 2019;(15):456–7.

[8] Pope JE, Johnson SR. New Classification Criteria for Systemic Sclerosis (Scleroderma). Rheum Dis Clin North Am. 2015;41(3):383–98.

[9] Sureshan D, Riyaz N, Thumbayil L. Cross-sectional study on clinical features and histopathology of systemic sclerosis. J Ski Sex Transm Dis. 2019;1(2):77–83.

[10] Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatology Venereol. 2017;31(9):1401–24.

[11] Sobolewski P, Ma?li?ska M, Wieczorek M, ?agun Z, Malewska A, Roszkiewicz M, et al. Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221–33.

[12] Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;6736(17):1–15.

[13] Showalter K, Gordon JK. Skin Histology in Systemic Sclerosis: a Relevant Clinical Biomarker. Curr Rheumatol Rep. 2021;23(3):1–8.

[14] Ravi D, Prabhu S. Management of Systemic Sclerosis: A Dermatologist’s Approach. Clin Dermatology Rev. 2019;3:34–40.

[15] Hachulla E, Agard C, Allanore Y, Avouac J, Bader-Meunier B, Belot A, et al. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis. 2021;16(Suppl 2):1–61.

[16] Fernández-Codina A, Walker KM, Pope JE. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheumatol. 2018;70(11):1820–8.

Limited Cutaneous Scleroderma

Abstract

References

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