Eponyms in syphilis.

Eponym has originated from the Greek word "eponymos" meaning "giving name". It is a tribute to the pioneers in the field who have significantly contributed towards present understanding of the subject. Syphilis has amazed and plagued mankind since eternity. This disease is a great masquerade and can humble best of physicians with its varied presentations. Keen and immaculate observations of these workers have eased our understanding of many signs and phenomenon associated with this disease. This has led to evolution of number of eponyms, perhaps no other disease has as many eponyms associated with it, as does syphilis. Eponyms such as Kassowitz's law, Clutton's joints, Higoumenaki sign, Argyll-Robertson pupil etc. help in providing easy milieu for remembering. Besides paying tributes to stalwarts in the field, who dedicated their lives for this cause, they also facilitated our current understanding of the great masquerade.

INTRODUCTION

An eponym is a word or name derived from the name of a person. It has originated from the Greek word “eponymous” meaning “giving name.” It is a tribute to the pioneers in the field who have significantly contributed toward present understanding of the subject.

Syphilis is known since antiquity; perhaps no other disease has amazed and plagued mankind as much as this malady. Across ages, physicians have dedicated their lives toward understanding of this disease and providing a panacea for this great masquerader. Lot of research has gone into this field over the years, keen and immaculate observations of these workers have eased our understanding of many signs and phenomenon associated with this disease. This has led to the evolution of a number of eponyms, perhaps no other disease has as many eponyms associated with it, as does syphilis has. Sir William Osler has aptly said “He, who knows syphilis, knows medicine.”

SYPHILIS

It has been known by various names viz Great Pox, morbus gallicus, lues venereum, Polish disease, etc. It is believed to have acquired its present name from the legend of mythical shepherd, syphilis, in poem, and Syphilis Sive Morbus Gallicus, described by Hieronymus Fracastorius in 1530, which means “Syphilis or the French Disease.”[1]

In hereditary transmission, nature plays some queer and unexplainable pranks. Three conditions are so well-known that they have come to be recognized as established laws: Kassowitz's, Colle's and Profeta's law.

KASSOWITZS LAW

Also called as Diday's law emphasizes rate of vertical transmission in untreated mother significantly drops with the passage of time. The longer the interval between infection and conception better is the outcome. It is believed that transmission rates drops from 70-100% to 10% during primary syphilis and late latent disease, respectively.[2]

COLLES’ LAW

Named after Colles also known as Baumιs law states that a child born to apparently asymptomatic mother with no signs of venereal symptoms, and presents with syphilis after few weeks would infect the healthiest nursemaid, but not its mother.[3] Colles at that time was ignorant that the mother already syphilis.

PROFETA'S LA

It states that an apparently healthy baby born of a syphilitic mother cannot be infected by her mother due to suckling or by a wet nurse.[4]

Hutchinson's triad is named after Sir Jonathan Hutchinson (1828-1913) British surgeon and pathologist. It is a classical triad characterized by the presence of interstitial keratitis, eighth nerve deafness, and Hutchinson's teeth.[5] Hutchinson's triad usually becomes apparent after 5-year of age. Hutchinson's teeth are peg-shaped incisors having a crescent-shaped notch in the cutting edge due to enamel hypoplasia, and occurring especially in children with congenital syphilis.

FOURNIER'S TEET

Also known as mulberry molars and moon molars. These result due to enamel hypoplasia of the first molar. Fournier described a less characteristic deformity of the first molar, which he said was even more common than Hutchinson's teeth.[6]

CLUTTON'S JOINT

First described by Clutton in 1886. It is arthritis in congenital syphilis in relatively older children. There is painless, symmetrical, joint swelling affecting the knees, presenting with synovitis, and joint effusions. It may also involve ankles, elbows, wrists, and fingers.[7]

PARROT'S PSEUDOPARALYSI

Also known as Parrot I syndrome or Bednar-Parrot syndrome - was first described by Parrot, Jules Marie, French physician, in 1871, this condition affected relatively younger children, within 8 months of birth in early congenital syphilis. Osteochondritis was the most common and the earliest lesion mainly affecting the upper limbs and knees. Pain in the extremities secondary to involvement of bone resulted in a lack of movement on the affected side.[8]

WIMBERGERS SIGN

Named after Heinrich Wimberger, German radiologist is also known as Wimberger's corner sign. It is a specific pathognomonic radiological sign of congenital syphilis in which there is localized bony destruction of the medial portion of the proximal tibia metaphysis.[9] Clinically, it manifests as mild irritability or pseodoparalysis. It should not be confused with Wimberger's ring sign seen in scurvy.

HIGOUMENAKI SIGN

Higoumenakis (1895-1983), a Greek dermatologist, student of Gaston Milian, a famous syphilologist was first to describe the enlargement of the sternal end of the (right) clavicle during late congenital syphilis. This sign has been suggested as an important diagnostic tool for screening congenital syphilis among the anthropological specimens.[10]

JARISCH-HERXHEIMER REACTION

Jarisch-Herxheimer reaction is described after Adolf Jarisch (1850-1902) an Austrian dermatologist and Herxheimer (1861-1942) a German dermatologist. Jarisch in 1895 and Herxheimer in 1902 observed that after the use of mercury for syphilis. This reaction is seen more frequently with the use of penicillins rather than other antibiotics. Early syphilis has the maximum chance of this reaction after treatment, followed by infants with congenital syphilis, pregnant patients with syphilis, and patients with neurosyphilis, respectively. Also known as “therapeutic shock,” results from the sudden excessive release of lipoproteins with inflammatory activities from dead or dying treponemes. It is associated with pyrexia, headache, malaise, myalgia, leukocytosis with lymphopenia within 12 h of initiation of treatment and terminates within 24 h.[11]

BUSCHKE OLLENDORFF SIGN

Jewish German dermatologist Abraham Buschke (1868-1943), and Helene Ollendorff Curth (1899-1982) German-American dermatologist observed the extreme sensitivity of secondary syphilitic lesions to pressure with a dull probe (e.g., papular lesions of syphilis) due to cutaneous vasculitis. It is also known as Ollendorff probe sign.[12]

DE GRAEFES RELAPSING CENTRAL RETINITIS

First described by Friedrich Wilhelm Ernst Albrecht von Graefe a pioneer of German ophthalmology in 1866, who named it as “relapsing central luetic retinitis.” It is circumscribed serous retinal detachment that is usually confined to the posterior pole caused by leakage of fluid through the retinal pigment epithelium. It is now known as central serous chorioretinopathy with largely unknown etiology.[13]

ARGYLL-ROBERTSON PUPIL

Named after Scottish ophthalmologist Douglas Moray Cooper Lamb Argyll Robertson (1837-1909) who died on January 3, 1909, Gonday near Mumbai, India. It is also known as reflex iridoplegia, and colloquially as “Prostitute's Pupil.” This is highly associated with neurosyphilis, and might also be seen with diabetic neuropathy. In this accommodation, reflex is preserved but pupillary reflex is compromised. It has been attributed to a dorsal midbrain lesion in neurosyphilis that interrupts the pupillary light reflex pathway but spares the more ventral pupillary near reflex pathway.[14]

JUSTUS'S TEST FOR SYPHILIS

Named after Justus. This test was used for diagnosis of syphilis before the advent of wasserman test. In this test change in hemoglobin level is determined before and 24 h after a single mercurial inunction. Initially, there is fall in hemoglobin by about 10-20% followed by a rise to a level above that which existed when the test was applied.[15]

December 1903, Volume 67, Issue 3, pp 363-386.

VIRCHOW'S SIGN

“The father of modern pathology” Rudolf Carl Virchow (1821-1902) observed that tongue often showed a smooth base in congenital syphilis. This is called as Virchow's sign.[16]

HUNTERIAN CHANCRE

Hunter (1728-1793) was a Scottish surgeon who believed in the Unity or Monist Theory, that is, gonorrhea and syphilis were the same disease. To prove his observation, he conducted a famous experiment in 1767, in which he inoculated himself with matter from a patient who suffered gonorrhea. Ten days later he developed a chancre, followed by secondary syphilis, thus proving his point. It is now believed that the donor had both syphilis and gonorrhea. Since then primary chancre is known as Hunterian chancre or hard chancre. He was later proved wrong by Philippe Ricord in 1838.[17]

RICORD'S CHANCRE

Philippe Ricord (1800-1889) was a French physician who described a syphilitic chancre with a thin parchment-like base. He conclusively proved that syphilis and gonorrhea were separate diseases. He also observed that bubo of syphilis was multiple, consisting of a chain of movable glands which are now called Glandulte Pleiades of Ricord. He is credited with categorized of syphilis into primary, secondary, and tertiary stages, a classification which has stood the test of time and is still in vogue.[18]

WASSERMANN TEST OR WASSERMANN REACTION

Named after the German bacteriologist August Paul von Wassermann (1866-1925), First diagnostic test of syphilis using blood serum or cerebrospinal fluid. It is a modification of the complement-fixation reaction.[19]

NELSON-MAYER TEST

Also known as Nelson's syphilis reaction or Nelson's treponemal immobilization test is named after Robert Armstrong Nelson and Manfred Martin Mayer. It is highly sensitive, the specific reaction for serodiagnosis of syphilis. It demonstrates of immobilizing antibodies in patients’ serum.[20]

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