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Eosinophilic fasciitis in a pregnant woman with corticosteroid dependence and good response to infliximab

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Abstract

Eosinophilic fasciitis (EF) is characterized by symmetrical thickening of subcutaneous muscular fascia, causing skin induration with wrinkles and prominent hair follicles: the classic peau d’orange. Eosinophilia is a characteristic—albeit not universal—finding. We present the case of a 43-year-old pregnant woman diagnosed with EF during pregnancy who had extensive cutaneous involvement and severe functional repercussions, including worsening of lung function and intrauterine growth restriction as a possible complication. Treatment with prednisone was initiated during gestation and it was necessary to increase the dose. After delivery, methotrexate treatment was initiated and the corticosteroid dose progressively decreased, with progressive worsening in the torso and abdomen and secondary dyspnea due to thoracic pressure. Treatment with infliximab was then initiated, with favorable progress, though residual ankle and tarsal joint stiffness and significant muscular atrophy in the limbs continued. The triggering factor of EF was not identified. In a systematic search of the medical literature, three cases of EF in pregnant woman without clear triggers were found. Interestingly, all three cases progressed favorably with steroid treatment. Apart from this case, there are only seven published cases of infliximab use in the literature, all with moderate or complete response. Infliximab could be an option for corticosteroid-dependent EF with no response to other options.

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Authors and Affiliations

  1. Internal Medicine Department, Hospital Costa del Sol, Marbella, Spain

    Nicolás Jiménez-García & Josefa Aguilar-García

  2. Dermatology Department, Hospital Costa del Sol, Marbella, Spain

    Inés Fernández-Canedo & Nuria Blázquez-Sánchez

  3. Pathology Department, Hospital Costa del Sol, Marbella, Spain

    Rafael Fúnez-Liébana

  4. Internal Medicine Department, Hospital Regional Universitario de Málaga, Málaga, Spain

    Carlos Romero-Gómez

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  1. Nicolás Jiménez-García
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  2. Josefa Aguilar-García
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All the authors whose names appear on the submission: (1) made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data; or the creation of new software used in the work; (2) drafted the work or revised it critically for important intellectual content; (3) approved the version to be published; and (4) agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Correspondence to Nicolás Jiménez-García.

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Jiménez-García, N., Aguilar-García, J., Fernández-Canedo, I. et al. Eosinophilic fasciitis in a pregnant woman with corticosteroid dependence and good response to infliximab. Rheumatol Int 41, 1531–1539 (2021). https://doi.org/10.1007/s00296-021-04787-6

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