Abstract 110: Histologic Assessment of a Case of Likely Kawasaki Disease in 1870 London
William Shrosbree, a 7 yo boy, died in 1870 at St. Bartholomew’s Hospital in London, England, “in consequence of scarlatinal dropsy” after a 2 d hospitalization. His case was cryptically reported briefly by Dr. Samuel Gee in 1871, who found 3 epicardial coronary artery (CA) aneurysms at autopsy, each containing “small recent clots, quite loose.” Two aneurysms were “pea-sized” at the apex close to the tip of the right auricular appendage, while the 3rd was the size of a “horse bean” and located in the sulcus at the posterior base of the ventricles. “Specks of atheroma” were described in the aorta “near the valves” and on the aortic cusp of the mitral valve. The heart was reported to be of normal size, and the valves were healthy. We have had the opportunity to study a limited number of CA photomicrographs of this specimen.
Overall, the vasculopathy in this case is highly compatible with our recent description of pathognomonic histologic features in 41 fatal or transplanted KD cases with severe disease (PLoS One, 2012). Of the 3 characteristic linked pathologic processes we reported, the limited number of sections do not show acute self-limiting necrotizing arteritis, which is relatively rare. Areas of subacute/chronic (SA/C) vasculitis containing barely recognizable lymphocytes, variably damaged media and prominent fibrotic adventitial fibrosis are seen. Very little of the internal and external elastic lanima could be seen in EVG and methylene blue stained sections. The most characteristic of the 3 processes, luminal myofibroblastic proliferation (LMP), is variable in prominence. The medium power magnification, H+E and methylene blue stained sections show a relatively uniform circumferential luminal layer thicker than the normal media. A higher magnification H+E section shows up to 100% LMP narrowing of the lumen involving about half the vessel, containing relatively prominent pleomorphic cells that may be myofibroblasts. The lumen in another section shows total obliteration by LMP with similar myofibroblasts and half of the media replaced by LMP.
The gross and histologic features of the CA of the child who died in 1870 in London are highly compatible with the changes noted in severe KD, suggesting that this illness was present as early as the 19th Century.
Author Disclosures: S.T. Shulman: None. A. Rowley: None. J. Orenstein: None.
- © 2015 by American Heart Association, Inc.