Thomas Chalopin1, Nicolas Vallet1, Marion Morel2, Raphael Maguet1, Louis d'Alteroche3, Gonzague de Pinieux4, Olivier Hérault5,6, Emmanuel Gyan1,6,7, Laurent Sutton1, Alban Villate8. 1. Department of Hematology and Cell Therapy, University Hospital of Tours, 2 Boulevard Tonnellé, 37044, Tours Cedex 9, France. 2. Department of Medical Imaging, University Hospital of Tours, Tours, France. 3. Department of Hepatology, University Hospital of Tours, Tours, France. 4. Department of Pathology, University Hospital of Tours, Tours, France. 5. Department of Biological Hematology, University Hospital of Tours, Tours, France. 6. Groupe Innovation et Ciblage Cellulaire EA 7501, ERL 7001 LNOx, CNRS-University of Tours, Tours, France. 7. Clinical Investigation Center, University Hospital of Tours-INSERM U1415, Tours, France. 8. Department of Hematology and Cell Therapy, University Hospital of Tours, 2 Boulevard Tonnellé, 37044, Tours Cedex 9, France. a.villate@chu-tours.fr.
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.
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.
Authors: T Kirchgesner; B Dallaudière; P Omoumi; J Malghem; B Vande Berg; F Lecouvet; F Houssiau; C Galant; A Larbi Journal: Diagn Interv Imaging Date: 2015-03-04 Impact factor: 4.026
Authors: C Romano; P Rubegni; G De Aloe; E Stanghellini; G D'Ascenzo; L Andreassi; M Fimiani Journal: J Eur Acad Dermatol Venereol Date: 2003-01 Impact factor: 6.166