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

• Published in: Rheumatology international Vol. 41; no. 8; pp. 1531 - 1539
• Main Authors: Jiménez-García, Nicolás, Aguilar-García, Josefa, Fernández-Canedo, Inés, Blázquez-Sánchez, Nuria, Fúnez-Liébana, Rafael, Romero-Gómez, Carlos
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-08-2021; Springer Nature B.V
• Subjects: Adult; Antirheumatic Agents - therapeutic use; Biopsy; Case Based Review; Drug dosages; Eosinophilia - drug therapy; Fasciitis - drug therapy; Female; Hepatitis; Hospitals; Humans; Infliximab - therapeutic use; Internal medicine; Leukemia; Light therapy; Medicine; Medicine & Public Health; Monoclonal antibodies; Pathology; Prednisolone - administration & dosage; Pregnancy; Pregnancy Complications - drug therapy; Rheumatology; Scleroderma; Skin; Skin - pathology; Steroids; TNF inhibitors; Tumors; Pregnancy; Intrauterine growth restriction; Infliximab; Methotrexate; Corticosteroid dependence; Eosinophilic fasciitis
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-021-04787-6

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.