Quiz Case #3
Morphea is a rare, fibrosing skin disorder caused by the overproduction of collagen by fibroblasts, resulting in a thickening of the dermis, subcutaneous tissue, underlyingbone, and rarely the central nervous system when present on the face and head. Morphea is clinically differentiated from systemic scleroderma based on the absence of sclerodactyly, Raynaud’s phenomenon, telangiectasias, gastrointestinal involvement, and nail-fold capillary changes.
Quantifying disease severity and activity is a major challenge in Localised scleroderma (LSc) and well established outcome measures for clinical trials are lacking. Clinical scores such as the modified Rodnan score, which is validated in systemic sclerosis has limited use in monitoring LSc. The Localised Scleroderma Assessment tool (LoSCAT) is under evaluation and comprises of an assessment of disease activity with assesment for skin damage.
Systemic therapy with agents like MTX, mycophenolate mofetil, D- penicillamine, and cyclosporine should be highly considered in children with progressive or extensive cutaneous disease with lesions affecting the face or overlying joints, increasing the risk of developing both physical deformity and fun
References:
-
Browning JC. Pediatric Morphea. Dermatologic Clinics 2013;31(2):229–237.
-
Reed AM, Lazzara DR, Skopit A. Pediatric Morphea: A Case report and Review of
Current Treatment Options. Semanticscholar. 2017:37-9.
-
Eleftheriou D, Shaw L. Morphea (Localized Scleroderms). In Hoeger PH, Kinsler V,
Yan A, editors. Harper’s Textbook of Pediatric Dermatology. 4th edition. Oxford:
Wiley-Blackwel; 2020;99:1175-82.
-
Bielsa MI. Update on the Classification and Treatment of Localized Scleroderma.
Actas Dermosifiliogr. 2013:104(8):654-66.
Author:
July Iriani Rahardja
MD
Department of Dermatology and Venereology, Karya Medika Hospital in Bekasi Indonesia.
Morphea is a rare, fibrosing skin disorder caused by the overproduction of collagen by fibroblasts, resulting in a thickening of the dermis, subcutaneous tissue, underlyingbone, and rarely the central nervous system when present on the face and head. Morphea is clinically differentiated from systemic scleroderma based on the absence of sclerodactyly, Raynaud’s phenomenon, telangiectasias, gastrointestinal involvement, and nail-fold capillary changes.
Quantifying disease severity and activity is a major challenge in Localised scleroderma (LSc) and well established outcome measures for clinical trials are lacking. Clinical scores such as the modified Rodnan score, which is validated in systemic sclerosis has limited use in monitoring LSc. The Localised Scleroderma Assessment tool (LoSCAT) is under evaluation and comprises of an assessment of disease activity with assesment for skin damage.
Systemic therapy with agents like MTX, mycophenolate mofetil, D- penicillamine, and cyclosporine should be highly considered in children with progressive or extensive cutaneous disease with lesions affecting the face or overlying joints, increasing the risk of developing both physical deformity and fun
References:
-
Browning JC. Pediatric Morphea. Dermatologic Clinics 2013;31(2):229–237.
-
Reed AM, Lazzara DR, Skopit A. Pediatric Morphea: A Case report and Review of
Current Treatment Options. Semanticscholar. 2017:37-9.
-
Eleftheriou D, Shaw L. Morphea (Localized Scleroderms). In Hoeger PH, Kinsler V,
Yan A, editors. Harper’s Textbook of Pediatric Dermatology. 4th edition. Oxford:
Wiley-Blackwel; 2020;99:1175-82.
-
Bielsa MI. Update on the Classification and Treatment of Localized Scleroderma.
Actas Dermosifiliogr. 2013:104(8):654-66.
Author:
July Iriani Rahardja
MD
Department of Dermatology and Venereology, Karya Medika Hospital in Bekasi Indonesia.