Dataset Information

Eosinophilic Fasciitis Presenting as an Ichthyosiform Eruption of the Bilateral Ankles.

ABSTRACT: Eosinophilic fasciitis (EF) is a rare connective tissue disease which closely resembles other scleroderma-like diseases. EF presents with painful swelling and hardening of the distal limbs and is often preceded by a history of strenuous exercise. The marked fascial fibrosis in EF can lead to joint contractures and causes significant morbidity in affected individuals. The authors present a rare case of EF presenting as an ichthyosiform eruption of the bilateral ankles with gradual improvement after the implementation of oral prednisone, hydroxychloroquine, and methotrexate.

SUBMITTER: Ivanov NN

PROVIDER: S-EPMC9984939 | biostudies-literature | 2023 Jan-Dec

REPOSITORIES: biostudies-literature

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Eosinophilic Fasciitis Presenting as an Ichthyosiform Eruption of the Bilateral Ankles.

Ivanov Nedyalko N NN Garvin Ashley A Mahon Michael J MJ Stephenson Sean S

Case reports in dermatology 20230101 1

Eosinophilic fasciitis (EF) is a rare connective tissue disease which closely resembles other scleroderma-like diseases. EF presents with painful swelling and hardening of the distal limbs and is often preceded by a history of strenuous exercise. The marked fascial fibrosis in EF can lead to joint contractures and causes significant morbidity in affected individuals. The authors present a rare case of EF presenting as an ichthyosiform eruption of the bilateral ankles with gradual improvement aft ...[more]

PMID: 36879682

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Project description:Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

Dataset Information

Eosinophilic Fasciitis Presenting as an Ichthyosiform Eruption of the Bilateral Ankles.

Publications

Eosinophilic Fasciitis Presenting as an Ichthyosiform Eruption of the Bilateral Ankles.

Similar Datasets

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