Eosinophilic fasciitis (Shulman syndrome), a rare entity and diagnostic challenge, as a manifestation of severe chronic graft-versus-host disease: a case report

Journal of Medical Case Reports

Olivier Hérault¹  Nicolas Vallet²  Alban Villate²  Thomas Chalopin²  Laurent Sutton²  Raphael Maguet²  Emmanuel Gyan³  Louis d’Alteroche⁴  Marion Morel⁵  Gonzague de Pinieux⁶ 

[1] Department of Biological Hematology, University Hospital of Tours, Tours, France;Groupe Innovation et Ciblage Cellulaire EA 7501, ERL 7001 LNOx, CNRS–University of Tours, Tours, France;Department of Hematology and Cell Therapy, University Hospital of Tours, 2 Boulevard Tonnellé, 37044, Tours Cedex 9, France;Department of Hematology and Cell Therapy, University Hospital of Tours, 2 Boulevard Tonnellé, 37044, Tours Cedex 9, France;Groupe Innovation et Ciblage Cellulaire EA 7501, ERL 7001 LNOx, CNRS–University of Tours, Tours, France;Clinical Investigation Center, University Hospital of Tours–INSERM U1415, Tours, France;Department of Hepatology, University Hospital of Tours, Tours, France;Department of Medical Imaging, University Hospital of Tours, Tours, France;Department of Pathology, University Hospital of Tours, Tours, France;

关键词: Multiple myeloma;    Allogenic hematopoietic stem cell transplantation;    Eosinophilic fasciitis;    Chronic graft-versus-host-disease;   

DOI  :  10.1186/s13256-021-02735-3

来源: Springer

【 摘 要 】

BackgroundShulman’s disease, or eosinophilic fasciitis (EF), is a rare autoimmune disease, characterized by sclerodermic skin lesions with progressive induration and thickening of the soft tissues. Chronic graft-versus-host-disease (GVHD) presenting as EF is a very rare manifestation of cutaneous GVHD.Case presentationWe report an unusual case of EF in a 46-year-old Caucasian male patient who had received an allogenic hematopoietic stem cell transplantation in the context of relapsed/refractory multiple myeloma. The diagnosis was challenging, with the patient presenting hepatic dysfunction, normal eosinophils count, and incomplete clinical signs. Magnetic resonance imaging (MRI) and skin biopsy confirmed the diagnosis ofEF. Early initiation of specific treatment with corticosteroids and prednisolone achieved complete response.ConclusionIn practice, incomplete signs in this rare complication should lead to MRI as it is a major tool to guide decision-making based on the skin biopsy, allowing a rapid diagnosis and the initiation of treatment without delay.

【 授权许可】

CC BY