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

• Main Authors: Thomas Chalopin, Nicolas Vallet, Marion Morel, Raphael Maguet, Louis d’Alteroche, Gonzague de Pinieux, Olivier Hérault, Emmanuel Gyan, Laurent Sutton, Alban Villate
• Format: Article
• Language: English
• Published: BMC 2021-03-01
• Series: Journal of Medical Case Reports
• Subjects: Multiple myeloma; Allogenic hematopoietic stem cell transplantation; Eosinophilic fasciitis; Chronic graft-versus-host-disease
• Online Access: https://doi.org/10.1186/s13256-021-02735-3

Abstract Background Shulman’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 presentation We 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 of EF. Early initiation of specific treatment with corticosteroids and prednisolone achieved complete response. Conclusion In 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.