Localized Chronic Fibrosing Vasculitis or Localized Erythema Elevatum Diutinum?

Localized chronic fibrosing vasculitis is a rare dermatosis that histologically resembles late‐stage erythema elevatum diutinum (EED) but has a different clinical presentation. A 62‐year‐old male presented with bilateral nodules on his heels that first appeared two years ago and over the past six months had become extremely painful. He denied any recent trauma to the sites, and his medical history was significant only for diabetes mellitus, coronary artery disease, and osteoarthritis. Physical exam demonstrated focally ulcerated violet‐red three‐centimeter nodules on the medial aspects of both heels. Biopsy revealed dense concentric and lamellar fibrosis with foci of leukocytoclastic debris and a sparse infiltrate of histiocytes, neutrophils, eosinophils, and lymphocytes. No granulomas were present, and histochemical stains and tissue cultures for microorganisms were negative. Imaging studies showed no evidence of underlying osteomyelitis, cellulitis, or abscess formation. Laboratory studies demonstrated a markedly elevated IgA level that was shown to be polyclonal on serum immunofixation studies. All peripheral blood cell counts were normal. Thorough evaluations for systemic vasculitides and connective tissue disorders were negative. A diagnosis of localized chronic fibrosing vasculitis was made. This case illustrates the clinicopathologic overlap between this disorder and EED.