Disabling pansclerotic morphoea of childhood

Jamalpur, Indirakshi; Mogili, Harikrishna Reddy; Koratala, Abhilash

doi:10.1136/bcr-2017-222132

Cited by 3 publications

(1 citation statement)

References 8 publications

(5 reference statements)

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“…As a result, contractures, large ulcerations and impaired wound healing are frequently described in association with this subtype. The disease usually starts in childhood and it is associated with important mortality, death causes including sepsis, gangrene and cardiopulmonary disease ( 76 , 77 ).…”

Section: Clinical Typesmentioning

confidence: 99%

Morphea: The 2023 update

et al. 2023

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Morphea, also known as localized scleroderma, is a chronic inflammatory connective tissue disorder with variable clinical presentations, that affects both adults and children. It is characterized by inflammation and fibrosis of the skin and underlying soft tissue, in certain cases even of the surrounding structures such as fascia, muscle, bone and central nervous system. While the etiology is still unknown, many factors may contribute to disease development, including genetic predisposition, vascular dysregulation, TH1/TH2 imbalance with chemokines and cytokines associated with interferon-γ and profibrotic pathways as well as certain environmental factors. Since the disease may progress to permanent cosmetic and functional sequelae, it is crucial to properly assess the disease activity and to initiate promptly the adequate treatment, thus preventing subsequent damage. The mainstay of treatment is based on corticosteroids and methotrexate. These, however, are limited by their toxicity, especially if applied long-term. Furthermore, corticosteroids and methotrexate often do not sufficiently control the disease and/or the frequent relapses of morphea. This review presents the current understanding of morphea by discussing its epidemiology, diagnosis, management and prognosis. In addition, it will describe recent pathogenetic findings, thus proposing potential novel targets for therapeutic development in morphea.

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Section: Clinical Typesmentioning

confidence: 99%

Morphea: The 2023 update

et al. 2023

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Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review

et al. 2019

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Disabling pansclerotic morphea in an adult Yemeni patient: a case report

Ali Alshami,

Al-shami,

Mohammad Alshami

2023

JDC

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Pansclerotic morphea is a highly rare, treatment-resistant, and debilitating variant of localized morphea that affects the entire thickness of the skin and sometimes involves muscles and bone. It manifests as expanding sclerotic plaques that eventually coalesce over the whole trunk or circumferentially extend into the extremities. It is also associated with an increased risk of cutaneous squamous cell carcinoma. Herein, we present the first case of a 30-year-old male patient with typical features of pansclerotic morphea in Yemen. Our patient’s case is unique because it presents a new sign not reported before, namely the boxer-jump sign, besides other previously reported typical signs.

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Disabling pansclerotic morphoea of childhood

Cited by 3 publications

References 8 publications

Morphea: The 2023 update

Morphea: The 2023 update

Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review

Disabling pansclerotic morphea in an adult Yemeni patient: a case report

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