Quiz Case #2


A 9-year–old boy presented to the clinic with complaints of dark brown plaques on his right abdomen and right thigh of eleven months duration. It had gradually progressed, hardened and resulted in painful and restriction of movement. There was no history of Raynaud’s phenomenon, dysphagia, myalgia, headache, or seizures.

A 9-year–old boy presented to the clinic with complaints of dark brown plaques on his right abdomen and right thigh of eleven months duration. It had gradually progressed, hardened and resulted in painful and restriction of movement. There was no history of Raynaud’s phenomenon, dysphagia, myalgia, headache, or seizures.
1. What is your most likely diagnosis? 
Systemic sclerosis
Eosinophilic fasciitis
Morphea
Linear lupus erythematosus panniculitis 
Psoriasis vulgaris
2. What clinical scores will you use for disease monitoring? 
Modified Rodnan scores
The Localised Scleroderma Assessment tool
Cutaneous LE Disease Area and Severity Index
Sistemic Lupus Erythematosus Disease Activity Index
Psoriasis Area and Severity Index 
3. What treatment do you suggest for the patient if there is physical deformity and functional impairment ?
Topical corticosteroid
Topical calcipotriol
Methothrexate 
Surgery
Psoralen NBUVB

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 functional impairment. According to a 2013 article by Bielsa, psoralen UVA or NBUVB should be initiated in patients with generalized morphea without joint contractures.

References:

  1. Browning JC. Pediatric Morphea. Dermatologic Clinics 2013;31(2):229–237.

  2. Reed AM, Lazzara DR, Skopit A. Pediatric Morphea: A Case report and Review of

    Current Treatment Options. Semanticscholar. 2017:37-9.

  3. 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.

  4. 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 functional impairment. According to a 2013 article by Bielsa, psoralen UVA or NBUVB should be initiated in patients with generalized morphea without joint contractures.

References:

  1. Browning JC. Pediatric Morphea. Dermatologic Clinics 2013;31(2):229–237.

  2. Reed AM, Lazzara DR, Skopit A. Pediatric Morphea: A Case report and Review of

    Current Treatment Options. Semanticscholar. 2017:37-9.

  3. 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.

  4. 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.


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