Writing guidelines without a net: ECD

Erdheim–Chester disease with unusual clinicopathological features complicated by DRESS syndrome, disseminated Cytomegalovirus infection and hemophagocytic lymphohistiocytosis

“…The recent decline in the hospital-based autopsy rates is likely to have a negative impact on the diagnosis of orphan diseases like ECD (27). Guidelines for ECD proposed by Diamond et al (28) based on the inadequate literature evidence along with a consensus of those involved in that conference are a double-edged sword (29). Guidelines are given from the consensus by a group of experts in that particular field, which are not always perfect and evolve over a time period through accumulation of additional evidence like the unusual presentation in the present case.…”