Morphea
Symptoms
Skin Findings
A localized and circumscribed cutaneous sclerosis characterized by early violaceous, later ivory- colored, hardened skin.
Plaques
Usually none.
No Raynaud phenomenon.
2 - 15 cm.
Linear and pansclerotic morphea assymetry, flexion contractures and disability.
Consistency and colour depends con the stage of the disease.
Epidemiology
Classification
Rare between the age of 20 and 50.
Pansclerotic
Generalized8%
Fronto-parietal3,5%
Linear6%
Cirncumscribed65%
Prevalence 1-3:100.000
Related Disorders
Etiology
Lichen Sclerosus.
Artropoderma of pasini and Pierini.
Mostly idiopatic.
Eosinophilic Fasciitis.
Classic Morphea results from Borrelia burgdorferi infection.
Differential Diagnosis
Morphea has been noted after x-irradiation for breast cancer.
Systemic Sclerosis.
Lipodermatosclerosis.
It is unrelated to systemic scleroderma.
Carcinoma en cuirasse and postirradiation morphea.
Morphea
Superficial limited morphoea
-Mild Topical therapy -Phototherapy (UVA1, nbUVB, PUVA)
Diagnosis
Clinical diagnosis, usually confirmed by skin biopsy.Borrelia Burgdoifei serology.Auto Antibodies serology. Immflamatory markers. Eosinophiles count.
Superficial generalised or adult-onset linear morphoea
-Moderate Topical therapies AND phototherapy Systemic therapy
Deep forms of morphoea and paediatric onset linear morphoea — severe
1. Methotrexate +/- systemic corticosteroids (IV pulsed &/or oral) 2. Mycophenolate mofetil +/- corticosteroids (IV pulsed &/or oral) 3. Combination therapies Combine 1st and 2nd line treatments Add hydroxychloroquine Add phototherapy (UVA1 or PUVA if available) Other options Ciclosporin, Abatacept, Tocilizumab, Extracorporeal photopheresis
Treatment
There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression.
Topical therapy
Phototherapy
Systemic treatment
Referrences
- DermNet NZ. Our Mission. https://www.dermnetnz.org/. Accessed January 25, 2021.
- Fett, Nicole & Werth, Victoria. (2011). Update on morphea. Part I. Epidemiology, clinical presentation, and pathogenesis. Journal of the American Academy of Dermatology. 64. 217-28.
- Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology, 8e. Klaus Wolff, Richard Allen Johnson, Arturo P. Saavedra, Ellen K. Roh.
- Dermatology essentials. In Bolognia, J., In Schaffer, J. V., In Duncan, K. O., & In Ko, C. J. (2014).
- Adamska, Urszula & Slinko, Anna & Białecka, Agnieszka & Męcińska-Jundziłł, Kaja & Adamski, Piotr & Czajkowski, Rafał. (2018). Unilateral generalized morphea: a case report and literature review. Postepy Dermatologii I Alergologii. 35. 425-428. 10.5114/pdia.2017.70258.
Morphoea
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Created on February 7, 2021
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Transcript
Morphea
Symptoms
Skin Findings
A localized and circumscribed cutaneous sclerosis characterized by early violaceous, later ivory- colored, hardened skin.
Plaques
Usually none.
No Raynaud phenomenon.
2 - 15 cm.
Linear and pansclerotic morphea assymetry, flexion contractures and disability.
Consistency and colour depends con the stage of the disease.
Epidemiology
Classification
Rare between the age of 20 and 50.
Pansclerotic
Generalized8%
Fronto-parietal3,5%
Linear6%
Cirncumscribed65%
Prevalence 1-3:100.000
Related Disorders
Etiology
Lichen Sclerosus.
Artropoderma of pasini and Pierini.
Mostly idiopatic.
Eosinophilic Fasciitis.
Classic Morphea results from Borrelia burgdorferi infection.
Differential Diagnosis
Morphea has been noted after x-irradiation for breast cancer.
Systemic Sclerosis.
Lipodermatosclerosis.
It is unrelated to systemic scleroderma.
Carcinoma en cuirasse and postirradiation morphea.
Morphea
Superficial limited morphoea
-Mild Topical therapy -Phototherapy (UVA1, nbUVB, PUVA)
Diagnosis
Clinical diagnosis, usually confirmed by skin biopsy.Borrelia Burgdoifei serology.Auto Antibodies serology. Immflamatory markers. Eosinophiles count.
Superficial generalised or adult-onset linear morphoea
-Moderate Topical therapies AND phototherapy Systemic therapy
Deep forms of morphoea and paediatric onset linear morphoea — severe
1. Methotrexate +/- systemic corticosteroids (IV pulsed &/or oral) 2. Mycophenolate mofetil +/- corticosteroids (IV pulsed &/or oral) 3. Combination therapies Combine 1st and 2nd line treatments Add hydroxychloroquine Add phototherapy (UVA1 or PUVA if available) Other options Ciclosporin, Abatacept, Tocilizumab, Extracorporeal photopheresis
Treatment
There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression.
Topical therapy
Phototherapy
Systemic treatment
Referrences