Abdominal Graft-versus-host disease and Sweet syndrome

Case contributed by Liz Silverstone
Diagnosis probable

Presentation

Emergency admission with febrile neutropaenia, severe abdominal pain and diarrhea.

Patient Data

Age: 40 years
Gender: Female

Extensive continuous small and large bowel wall-thickening, submucosal edema and mucosal hyperenhancement with prominent mesenteric vessels.

Irregular thickening of the distal abdominal aortic wall from the IMA origin to the bifurcation causing irregular luminal narrowing.

Increased attenuation in the subchondral bone of the superior femoral heads, with a convex-inferior margin.

Case Discussion

Aortitis and chronic femoral head AVN in a young adult prompted investigation of her previous history.

Allogeneic stem-cell transplant for recurrent AML was performed approximately one week earlier. Extensive small bowel edema and target appearance with or without colonic involvement is a feature of GVHD 1.

Several months previously she developed a rash which was diagnosed as leucocytoclastic vasculitis, (Sweet syndrome), which sometimes occurs as a paraneoplastic phenomenon.

Large vessel arteritis has been described as a rare manifestation of Sweet syndrome 2 and also can rarely occur in GVHD 3 following allogeneic stem cell transplant. Without access to previous imaging, it is impossible to plot the time-course of the vasculitis and the etiology is uncertain. FDG-PET CT can be used to determine the extent of a metabolically active vasculitis. Treatment is with IV methylprednisolone.

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