CASE 36
By: Dr. Waleed Althobaity MD
HISTORY: 40-year-old female known case of leukemia, post allogeneic-stem-cell-transplantation presented with chronic myalgia.
What are your findings?
What is your differential diagnosis?
What is your diagnosis?
FINDINGS:
Axial T2 FS (A), Axial T1 (B) and Coronal T2 FS weighted MR images of bilateral thighs demonstrate diffuse symmetrical bilateral deep fascial thickening and edema of high T2 and iso T1 signal intensity to muscles indicating fasciitis. No intramuscular edema on T2 images or fatty atrophic changes on T1 image to suggest myositis.
DIFFERENTIAL DIAGNOSIS:
Graft-Versus-Host Disease.
Eosinophilic fasciitis (Shulman syndrome).
Dermatomyositis.
Necrotizing fasciitis.
DIAGNOSIS:
Graft Vs. host disease.
PEARLS & DISCUSSION:
GVHD is still a major cause of morbidity and mortality after Allo-SCT and is caused by an immunological reaction against antigens in the SCT recipient by the immunocompetent donor graft. Muscle-related complications, fasciitis and myositis are relatively rare manifestations, and their clinical features resemble autoimmune eosinophilic fasciitis and idiopathic polymyositis. MRI manifestations can be as fasciitis (thickening and edema of fascia) with or without polymyositis (diffuse muscles edema). The diagnosis of GVHD is a combination of clinical symptoms, MRI findings of fasciitis and polymyositis +/- biopsy. MRI plays an important role in the disease extension and treatment response assessment.
In the cases of isolated fasciitis with no muscles involvement as in this case, the main DD are eosinophilic fasciitis and GVHD. Otherwise the muscles and subcutaneous involvements can be seen similarly in the other non-necrotizing inflammatory fasciitis such as dermatomyositis.
Eosinophilic fasciitis will show similar MRI manifestation of diffuse symmetrical fasciitis. It is a rare inflammatory condition in which superficial muscle fascia collagen is infiltrated by lymphocytes, plasma cells, and eosinophils. It usually presents in the extremity and trunk with scleroderma-like dermal induration and swelling. Laboratory findings include increased ESR, hyperglobulinemia, and peripheral eosinophilia are usually present.
Don’t forget to mention Necrotizing fasciitis in the DD in the case of fascial and muscles edematous changes. It is a medical emergency with potential lethal outcome. Dissecting gas along fascial planes in the absence of penetrating trauma (including iatrogenic) is essentially pathognomonic. However, the lack of soft-tissue emphysema does not exclude the diagnosis. It is mimic non-necrotizing inflammatory fasciitis. However, the clinical symptoms and the symmetrical bilateral involvement in this case are against necrotizing fasciitis.
FURTHER READINGS:
Musculocutaneous Chronic Graft-Versus-Host Disease: MRI Follow-Up of Patients Undergoing Immunosuppressive Therapy Marius Horger1, Wolfgang Bethge, Andreas Boss, Michael Fenchel, Claus D. Claussen, Marc Schmalzing and Wichard Vogel.
Eosinophilic fasciitis: spectrum of MRI findings. By Moulton SJ1, Kransdorf MJ, Ginsburg WW, Abril A, Persellin S.
Fasciitis and myositis: an analysis of muscle-related complications caused by chronic GVHD after allo-SCT. K Oda at el.
Necrotizing Fasciitis and Its Mimics: What Radiologists Need to Know. Ammar A. Chaudhry , Kevin S. Baker, Elaine S. Gould and Rajarsi Gupta.
