A clinical case of a patient with scleroderma-like syndrome in chronic graft-versus host disease


сhronic graft-versus host disease
allogenic haematopoietic stem cell transplantation
scleroderma-like syndrome

How to Cite

Parvova, I., Taneva, V., Ivanova-Todorova, E., Kyurkchiev, D., Kolarov, Z. and Hristov, E. 2019. A clinical case of a patient with scleroderma-like syndrome in chronic graft-versus host disease. Revmatologiia (Bulgaria). 27, 1 (Apr. 2019), 44-52. DOI:https://doi.org/10.35465/27.1.2019.pp44-52.


Chronic graft versus host disease is observed within the fi rst 100 days following allogeneic haematopoietic stem cell transplantation and can affect all tissues and organs (in 80% of the cases, it affects the skin). There are some clinical correspondences between chronic graft versus host disease and certain autoimmune diseases, such as systemic scleroderma, Sjogren‘s syndrome, autoimmune hepatitis. We present a case of a 54-year-old man with manifested diffuse, scleroderma-like skin changes, which occurred about a year and a half after allogenic bone marrow transplantation from an unrelated donor due to a blast transformation of chronic myelomonocytic leucosis type 2. The patient was treated in a haematology clinic with corticosteroids, 10 photophoresis sessions, Ciclosporin, Tacrolimus, Mycophenolate mofetil, Imatinib. It has been assumed that this is a case of chronic graft versus host disease resistant to corticosteroids, and Methotrexate 25 mg/weekly every other month was prescribed with no significant clinical improvement. In terms of the differential diagnosis, the question remains whether or not this is a case of paraneoplastic systemic scleroderma – autoimmune phenomena accompanying malignancy and often preceding it for months.



  1. Min CK. The pathophysiology of chronic graft-versus-host disease: the underveiling of an enigma. Korean J Hematol 2011; 46: 80-7.
  2. Foti R, Leonardi R, Rondinone R et al. Scleroderma-like disorders. Autoimmunity Reviews 2008; 7: 331-339.
  3. Schaff er JV, McNiff JM, Seropian S et al. Lichen sclerosus and eosinophilic fasciitis as manifestation of chronic graft- versus host disease: expanding the sclerodermoid spectrum. J Am Acad Dermatol 2005; 53: 591-601.
  4. Ratanatharathorn V, Ayash L, Lazarus HM et al. Chronic graft-versus-host disease: clinical manifestation and therapy. Bone Marrow Transplant 2001; 28: 121-9.
  5. Patriarca F, Skert C, Sperotto A et al. The development of autoantibodies after allogeneic stem cell transplantation is related with chronic graft-vs-host disease and immune recovery. Exp Hematol 2006; 34(3): 389-96.
  6. Klippel JH, Stone JH, Croff ord LJ, White, PH. Primer on the rheumatic diseases. N Y: Springer, 2008: 351.
  7. Conrad K, Schöbler W, Hiepe F, Fritzler MJ. Autoantibodies in Systemic Autoimmune Diseases. A Diagnostic Reference. 2, third Edition. Leipzig: Pabst science publishers, 2015.
  8. Holmes JA, Livesey SJ, Bedwell AE et al. Autoantibody analysis in chronic graft-versus-host disease. Bone Marrow Transplant 1989; 4: 529-531.
  9. Canninga-van Dijk MR, Van der Straaten HM, Fijnheer R et al. Anti-CD20 monoclonal antibody treatment in 6 patients with therapy-refractory chronic graft-versus-host disease. Blood 2004; 104: 2603-2606.
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