Some disease entities have such a characteristic and striking appearance that their written descriptions are unmistakable. One such disease is serpiginous choroiditis, an idiopathic choroiditis that was given its name in 1970 by Gass,1 who had described the disease earlier in 1967.2 It is an acute and chronically recurrent inflammation of the inner half of the choroid and retinal pigment epithelium that secondarily affects the retina. It typically starts in the peripapillary region and spreads centrifugally over months to years in episodes of inflammation that arise from the edge of previously involved choroid. The term serpiginous, which has been used extensively in the vocabulary of dermatology, was used by Gass to describe the apparently serpentine, creeping pattern of progressive pathologic changes of the fundus, as it was thought initially that the disease crept with a snake-like pattern at varying speeds from the peripapillary region. While we now know that the long-term pattern of progressive elongation of the large, irregularly shaped lesions are caused by recurrent episodes of inflammation with very little creeping of the lesions, the serpiginous appearance could not be denied, and the name has remained. Interestingly, we came across an article in the Archives of Surgery (London) entitled “Serpiginous Choroiditis in Scrofulous Subjects: Choroidal Lupus.”3 This article describes patients with a similar clinical syndrome, and it includes a reproduction of a fundus painting bearing a striking resemblance to what Gass named serpiginous choroiditis (Figure). The author of this article3 was Jonathan Hutchinson, an expert in, among other fields, dermatology, ophthalmology, syphilogy, leprosy, tuberculosis, and natural history.4 In addition to writing this article,3 Hutchinson was the editor and sole contributing author to the 11-volume run of the Archives of Surgery (London), which surprisingly has virtually no content on surgery but concerns itself mostly with the earlier mentioned subjects. Volume 11, the final volume and that which contains the article3 of interest, was dated 1900 but was probably not released until 1911, as Hutchinson had held it back hoping to announce continuation of the project. Hutchinson had, in fact, described cases of serpiginous choroiditis earlier in his volume on syphilis in 18875 and in his atlas of clinical illustrations.6 Thus, we have found that, astonishingly, this disease was described independently by 2 physicians using identical terminology but separated by 80 years of time. Figure. View LargeDownload A, Illustration of serpiginous choroiditis. (Reprinted from Hutchinson, “Serpiginous Choroiditis in Scrofulous Subjects: Choroidal Lupus.” Arch Surg [London]. 1900;11:126-135.) B, Funduscopic photographs from patients with serpiginous choroiditis (provided by J. Donald M. Gass, MD). Most striking in Hutchinson’s article is his careful description of the fundus appearance and the terminology that he uses. He described a disease in young, otherwise healthy patients that was characterized by “large creeping patches of atrophic denudation in the central parts of the fundus” that have “the appearance of inflammatory action at the border” and are crossed by retinal vessels that appear to be unaffected.3 In naming and describing the disease, he used the term serpiginous, borrowing the dermatologic term because he believed these lesions to be similar to serpiginous skin lesions seen as a manifestation of lupus. Hutchinson used geographic analogies to describe the findings in the fundus, noting that for 1 patient, “the edge [of the lesion] was sinuous, like the border of a continent in a map representing headlands and bays.”3 Later, he described another patient’s lesion, noting that “it is very remarkable how long and irregular in shape some of the patches were (like the North American lake-system).”3 Interestingly, Hutchinson’s cartographic allusions were not unique. Though the term serpiginous choroiditis as coined by Gass has been accepted, the condition had other names such as geographical choroidopathy7 and geographic helicoid peripapillary choroidopathy8 around the time of Gass’ description and naming of the disease. It truly is striking that, in their identification of serpiginous choroiditis, 4 physicians independently used similar terminology,1,3,7,8 and 2 people even gave the disease an identical name with an 80-year separation in time. This coincidence speaks to the distinctiveness of this disease as well as to the rich tradition of medicine. Even though Hutchinson’s text was unknown and his description of the condition forgotten, the terminology he used was so apt that 4 generations later, the same words were chosen to name it. Back to top Article Information Correspondence: Dr Wenick, Department of Ophthalmology, College of Physicians and Surgeons, Columbia University, 635 West 165th St, Room 316, New York, NY 10032 (firstname.lastname@example.org). Financial Disclosure: None. Acknowledgment: We would like to thank J. Donald M. Gass, MD, for his correspondence regarding his description and naming of serpiginous choroiditis and for providing us with images of the disease process. References 1. Gass JDM Stereoscopic Atlas of Macular Diseases: A Funduscopic and Angiographic Presentation. St Louis, Mo CV Mosby Co1970; 2. Gass JD Pathogenesis of disciform detachment of the neuroepithelium. Am J Ophthalmol 1967;63 ((suppl)) 1- 139PubMedGoogle Scholar 3. Hutchinson J Serpiginous choroiditis in scrofulous subjects: choroidal lupus. Arch Surg (Lond) 1900;11126- 135Google Scholar 4. Chance B Sir Jonathan Hutchinson, the greatest “generalized specialist” and his contribution to ophthalmology. Arch Ophthalmol 1935;14203- 228Google ScholarCrossref 5. Hutchinson J Syphilis. Philadelphia, Pa Lea Brothers1887; 6. Hutchinson J A Smaller Atlas of Illustrations of Clinical Surgery. London, England West Newman & Co1895; 7. Hamilton AMBird AC Geographical choroidopathy. Br J Ophthalmol 1974;58784- 797PubMedGoogle ScholarCrossref 8. Schatz HMaumenee AEPatz A Geographic helicoid peripapillary choroidopathy: clinical presentation and fluorescein angiographic findings. Trans Am Acad Ophthalmol Otolaryngol 1974;78OP747- OP761Google Scholar
Archives of Ophthalmology – American Medical Association
Published: Nov 1, 2005
Keywords: geographic helicoid peripapillary choroidopathy
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