Back to Search
Start Over
Chronic graft-versus-host disease presenting as eosinophilic fasciitis: therapeutic challenges and an additional case.
- Source :
-
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases [J Clin Rheumatol] 2015 Mar; Vol. 21 (2), pp. 86-94. - Publication Year :
- 2015
-
Abstract
- Chronic graft-versus-host disease (cGVHD) is one of the main late complications of allogeneic hematopoietic stem cell transplant and a major contributor to the mortality and morbidity in surviving recipients. Skin is the most common involved organ in cGVHD and may mimic a wide spectrum of dermatological conditions in its clinical and histopathologic manifestations. Some of the commonly simulated diseases are scleroderma, morphea, and lichen sclerosus. Chronic GVHD simulating eosinophilic fasciitis (EF) is relatively rare, frequently presenting with skin induration, a typical "peau d'orange" appearance, peripheral blood eosinophilia, myalgia, arthralgia, and arthritis leading to joint contractures in severe cases.Diagnosis is based on clinical manifestations and histopathology. Treatment is challenging because most cases are refractory to first-line therapy of glucocorticoids and calcineurin inhibitors (CNIs), and there is no standard second-line therapy.We report a comprehensive review of literature on all reported cases of CGVHD presenting as EF. We also describe an additional interesting case of cGVHD presenting as EF that was resistant to traditional therapy of high-dose glucocorticoids and cyclosporin A, but showed complete resolution of skin manifestations after addition of imatinib.Chronic GVHD presenting as EF is a rare variant of sclerodermatous cGVHD. Diagnosis is difficult, and treatment of cGVHD mimicking EF remains a therapeutic challenge because of obscure pathogenesis and poor response to traditional immunosuppressive medications. Emerging insights into the pathogenesis of cGVHD have resulted in the development of novel targeted therapies, which may improve outcomes and should be attempted in this subset of the disease. Larger studies are warranted to substantiate these preliminary findings.
- Subjects :
- Adult
Chronic Disease
Eosinophilia therapy
Fasciitis therapy
Graft vs Host Disease therapy
Humans
Male
Eosinophilia diagnosis
Eosinophilia etiology
Fasciitis diagnosis
Fasciitis etiology
Graft vs Host Disease diagnosis
Graft vs Host Disease etiology
Hematopoietic Stem Cell Transplantation adverse effects
Hodgkin Disease therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1536-7355
- Volume :
- 21
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
- Publication Type :
- Academic Journal
- Accession number :
- 25710860
- Full Text :
- https://doi.org/10.1097/RHU.0000000000000212