Case of Shulman Syndrome Highlights Importance of MRI in cGVHD Treatment Decisions

A case report of a patient with eosinophilic faciitis (EF), or Shulman syndrome, as a complication of chronic graft-versus-host-disease (cGVHD) underscores the importance of magnetic resonance imaging (MRI) as a tool to guide decision-making in similar cases.

According to the authors of the case report, MRI should be leveraged in the case of incomplete signs of the rare complication to help guide the skin biopsy, as MRI can help facilitate a rapid diagnosis and initiation of treatment.

The authors describe the case as unusual, with the patient presenting with EF in the absence of hypereosinophilia after receiving an allogenic hematopoietic stem cell transplant (ASCT) to treat his relapsed/refractory multiple myeloma.

“The incidence of EF in patients who have undergone ASCT presenting as cGVHD is rare,” emphasized the researchers. “To our knowledge, only one case of a patient with multiple myeloma has been reported.”

While not required for diagnosis, between 60% and 90% of cases present with eosinophilia. The majority (90%) of these cases also have skin involvement with an evolutive natural history. In the present case, the patient showed similar manifestations, exhibiting pain, swelling, induration, and edema.

The patient, a White male aged 46 years, also presented with hepatic dysfunction, which preceded the clinical signs, as well as normal eosinophil counts and incomplete clinical signs.

Based on body MRI results—which showed heightened signal intensity in short tau inversion recovery sequences in the superficial and deep fasciae—the patient received a skin-to-muscle biopsy of the left thigh. The biopsy confirmed fasciitis and edema with rare eosinophil infiltration.

“MRI is an important diagnostic and prognostic tool of EF, typically showing increased signal intensity within the fascia,” wrote the researchers. “It is the ideal imaging modality both for selecting the best site for biopsy and monitoring the course of EF following initiation of treatment.”

They continued, “In our case, MRI was crucial in enabling an early diagnosis in combination with other data (clinical, biological and anatomical pathology). Although not strictly required for diagnosis, MRI results appear to be required as part of the initial assessment of EF.”

Following the skin-to-muscle biopsy, liver biopsy confirmed the patient had accompanying cGVHD with fibrosis, and inflammatory infiltrate featuring lymphocytes and eosinophil granulocytes without apoptotic bodies.

Reference

Chalopin T, Vallet N, Morel M, et al. Eosinophilic fasciitis (Shulman syndrome), a rare entity and diagnostic challenge, as a manifestation of severe chronic graft-versus-host disease: a case report. J Med Case Rep. Published online March 15, 2021. doi:10.1186/s13256-021-02735-3