pediatric housecalls Robert R. Jarrett M.D. M.B.A. FAAP

Numbered Diseases of Childhood: FOURTH Disease – Filatov-Dukes’ Disease

April 3, 2014

We’ll continue our chats about “the Numbered Diseases of childhood” by discussing today FOURTH Disease and the unique circumstances about this number.

You remember from our previous discussions that the physicians in 1905 tried to streamline all the names of the rash-causing diseases by giving them numbers, one through six – oh, for the days!

The issue with ‘ol number four is that we… ah… sort of… er… “misplaced” it.

Oh, we know the name that it came from: Filatov-Dukes disease, named after two hugely famous pediatricians; but, all the jockeying and jostling in the subsequent years has… sort of… made us loose the disease that it went to.

[Which is why I can’t show you pictures, there are none.]

FOURTH Disease – Filatov-Dukes Disease

You need to bear with me a bit here for the recounting of this tale takes a bit of background information. We’re in the mid 1800’s. Doctors were real diagnosticians back then, out of necessity.

There were no MRIs or electron microscopes or viral identification kits or DNA analysis or serotyping. No antibiotics as we know it and even the actual nature of diseases themselves were just barely beginning to come to light.

Diagnoses were made by actually taking time to “talk” to patients and learn their history; then, by performing a meticulous physical exam and noticing everything. There was only the most rudimentary of laboratory testing, next to no effective treatments – and lots and lots of deaths.

Medicine In 1850’s

Medical knowledge was so tenuous that quacks and charlatans continually confused and bilked the lay public with wild claims (actually, much as they do today). Categorization of diseases was very, VERY general, such as: “rash-producing” or “non-rash-producing.”

This was a time when the way to discover a new disease was to obsessively and meticulously scrutinize every aspect of hundreds of children’s illnesses and hopefully find different sub-groups that could be lumped together, like: “rashes that start from the head downward” and those that “start on the whole body at once”; or, those that seemed “highly contagious” and those that were “less contagious”; or, those that “lasted 3 days” and those that “lasted for 7 days.”

That was exactly what was happening with rash diseases. Physicians were completely in the dark about the causes of most diseases. They knew about poxes – both “small” and “chicken”; and about plague, puerperal fevers and many other things. AND they knew about illnesses which were contagious between people, caused fevers with other complaints and which, before they were through, caused the skin to break out in red bumps and blotches — Rashes.

The term “Measles” had been coined but not really solidified into a known, certain diagnosis with a proven cause – like the luxury of viral sero-typing affords us today. The “hard measles” [FIRST disease] had such a well defined course and presentation that it was hard to confuse. “Scarlet Fever” [SECOND disease] was shown, in the 1700’s, to be “different” even though “similar” in many ways.

That had left another confusing similar rash which, in 1881, had just been separated out of the lump of exanthem-causing-diseases by brilliant pediatricians, who were none-the-less not yet brave enough to break out of the “measles” nomenclature so called it: Rubella – “little measles,” German measles.

Nil Fyodorovich Filatov (1847 – 1902)

This “Rubella” was clearly different than the “measles” of FIRST disease; but, was nowhere near as “cleanly” diagnosed. The rash at times began as minute dots on the face and neck then progressed down the body like “normal” measles; or, it could begin as a patchy erythema which appeared on the body all at once. Sometimes a patient felt ill, sometimes they barely knew they were sick. Sometimes the rash lasted for 3 days, sometimes for up to seven.

Enter Nil Fyodorovich Filatove, the “founder of Russian pediatrics” who went on to describe infectious mononucleosis (Filatov’s disease) in 1887 and introduce the serumal treatment of diphtheria in 1894. The guy was such a meticulous physical diagnostician he was the first to even notice the miniscule Koplik’s spots inside the mouth’s of measles’ patients (1895).

He astutely noticed that many of his patients with the “mild measles” (rubella) seemed to contract it twice – something that even back then they realized shouldn’t happen if it were caused by a virus (which they also didn’t yet know about).

Although medicine still didn’t yet understand the differences between viruses, bacterial infections and the immune response, they did know that people didn’t get “hard measles” twice; and if they did, it usually meant it was a different disease and one of the diagnoses were wrong.

This new “Rubella thing” just seemed to be acting much too squirrely for the diseases that they knew. Kids were not only getting FIRST, SECOND and THIRD diseases (hard measles, scarlet fever and Rubella)… but THIRD disease twice!

In 1885 he proposed that there were “two forms of German measles” and that they should be called “rubeola morbillosa” and “rubeola scarlatinosa” which, unexpectedly to him, caused quite a stir in the medical community.

First, few physicians of the time were as meticulous as he was, so they wouldn’t recognize a new disease even if it walked up and barked at them; and second, even though diseases back then did spread tremendously within communities, travel was much less, so not all communities had the same diseases. (The diseases that did cross community boundaries were so virulent that they wiped out whole percentages of the world’s population at once.)

Clement Dukes (1845-1925)

Fortunately, seven years later in 1892, an epidemic of these two confusing diseases hit the Rugby School in England where, through sheer volume, another physician, Clement Dukes, noticed the same thing; although, he didn’t realize and write about it until 1894. His paper first contrasted Measles and Scarlet Fever and then went on to draw a similar differentiation between the two diseases of the “Rubella variety” – the “measles type” and the “scarlet fever type.”

Dukes was a well known English pediatrician, son of a clergyman, working in prestigious hospitals until he was appointed medical officer at the huge Rugby school in 1871. He became world-renowned for a paper about the physical examination of 1000 boys at the school as well as his books and articles on schoolboy health.

By 1900 he had seen three outbreaks (1892, 96 and 1900) of this “scarlet fever variety” of rubella at the school, enough for him to publish copious findings and officially propose that Filatov had been vindicated and it should be recognized as “fourth disease.” Just like Filatov, his paper was met with both praise and derision – from people who had seen what they were talking about and those who hadn’t.

From then on the Filatov-Dukes’ or fourth disease did appear in the major pediatric textbooks; but, always with a footnote that its existence hadn’t been definitively proven – political kiss-of-death number one.

Symptoms and Diagnosis

Both Filatov and Dukes continued to see occasional outbreaks of “Fourth Disease” as long as they practiced. Others would occasionally be faced with epidemics of the same nature, slap their foreheads and write about “now we see what they meant by Fourth Disease” only to meet the same fate from the nay-sayers who claimed it didn’t exist because they had never seen it so it “must just be a mis-diagnosis of scarlet fever or something.”

Somewhere before 1910 Dr. v. Bokay wrote an extensive summary of his own experience and suggested that the disease be called Filatov-Dukes’ Disease. Additionally, a Dr. Unruh detailed extensively those patients in his practice that he had first-hand witnessed to have had prior rubella and scarlet fever and then contract FOURTH disease.

His painstakingly thorough notes give great detail:

Contagiousness – Decidedly, but less than either measles or scarlitina. Transmissibility, less than 14 – 21 days.
Incubation – 9-21 days, one case exactly 15 days. Longer than scarlatina’s 3-5 days.
Prodrome – Absent in many; severe cases have low fever, malaise, headache, anorexia, lassitude and muscle aches. Vomiting, rare.
ENT – No symptoms in many; occasionally, slight reddening of mucous membranes, very slight runny nose and trivial pinkness in the conjunctiva.
Rash – 24-36 hours after infection, commencing on face to total body within hours. Minutely punctate spots, smaller than rubella and less raised than measles. First diffuse, then coalesce into patches bound by an erythematous blush.

On the face, scarlet stain with irregular edges containing holes of un-invaded parts especially around nose and mouth. No predilection for lower abdomen, inner thighs and axillary folds like scarlet fever has. Mainly on backs of arms and is commonly profuse over the back and nates. More the color of scarlet fever and not ‘brownish’ like measles or rubella. Is NOT hot like scarlatina, rarely any itching or discomfort.
Rash Disappearance – Bright color until rapidly disappears within hours in 2-3 days, unlike either measles or scarlatina.
Desquamation – Usually has a fine, branny, desquamation over less than 2 weeks.
Temperature – Usually absent, unrelated to amount of rash, subsides with onset of rash, feels well, kept in bed with difficulty.
Pulse – Rare effect, only related to rare fever. Unlike scarlatina.
Tongue and Mouth – Almost none, No strawberry tongue, No desquamation, No Koplik’s spots.
Lymph Glands – Very slight, if any. Unlike either rubella or scarlet fever.
Course, Complications, Sequelae – Mild, don’t feel ill, won’t stay in bed, no complications or sequelae observed.

US Public Health Report

In 1910 all of this, including information from every published account, was integrated into an official US Public Health Report by Dr. Schereschewsky. He listed all of the above mentioned findings and gave information on how FOURTH disease could be differentiated from Scarlet Fever and Rubella.

His findings were that “It would seem to be established beyond a reasonable doubt that the fourth disease complies with those conditions which entitle it to a place in the catalogue of the eruptive fevers.”

The rash was different than either SECOND or THIRD disease, the length of the course was different, the glands were different, the peeling was different AND the infectivity was different. At the time, people with scarlatina or Rubella were kept quarantined – often at significant financial loss – until well after the rash had gone. That meant that the portion of FOURTH disease cases which were erroneously confused with them could be released to school and work earlier.

In spite of the evidence, and claiming to worry about the possible consequences to public health, he recommended that: “It would seem better, for the present at least, to leave the question of the FOURTH disease… (until) settled once and for all, and in the meantime to treat all mild scarlatinaform exanthems as scarlet fever” – political kiss-of-death number two.

The Decline and Fall

The venerable doctor Filatov died in Russia in 1902 and doctor Dukes retired in the twenties and died in 1925. Neither of them lived long enough to see the advances in microbiology or virology necessary to solidly establish the causative factor of their namesake disease – the one thing the naysayers seemed to demand of them.

The practice of medicine itself was changing. With them gone, few physicians remained who were punctilious enough to even ascertain an illnesses exposure timeline, the body-part predilection of a rash or any of the other subtle differences necessary to “split-the-hairs” required for the correct differential diagnosis. Especially since there was little difference gained in the treatment; therefore, giving little incentive to do so.

The disease itself didn’t help matters much. Even though highly contagious and causing a rash, nobody felt really bad, got any better with whatever treatment was used or had any consequences for getting it.

By 1945 the production of penicillin was enough to begin wiping out much of Scarlet Fever, which had been shown to be caused by a toxin produced by the streptococcus bacteria – not a virus at all. Cases of measles and rubella took a nose dive in 1963 and 69 respectively when wholesale vaccination programs were instituted to eradicate them and the mayhem they caused.

Kiss-of-death numbers 3,4,5,6, 7 and 8.

The Final Coffin Nails

After the deaths of its initial discoverers and the “back-seat” that rash diseases took in medicine following the demise of their leading causative agents (not to mention that the number of diseases so rapidly increased that the numbering system was abandoned) references to the FOURTH disease were all but removed from medical textbooks.

Once that happened it wouldn’t be long before some yay-hoo new doctor would write a paper about some disease clear out in left field and think he had resurrected the FOURTH disease. Such as in 1979 when a Doctor Powell enhanced the discussion about his new paper by claiming that Staphylococcal Scalded Skin Syndrome (SSSS) was FOURTH disease.

Never mind that SSSS is caused by a toxin produced by staphylococcus, usually from a wound or rash that looks absolutely nothing like anything previously mentioned, the patient is often sick to the point of toxicity and if minimally touched their skin falls off in large sheets with lots of secondary infections, scars and sequelae!

The mere thought that poor old doctors Filatov and Dukes were such poor diagnosticians that they couldn’t have told the difference between what they saw and wrote down and a patient with SSSS is to me a bit absurd.

Then, when the internet leaked out of the scientific community in 1994, it wouldn’t be long before writers, looking for a story without much effort and fact verification, would perpetuate the Powell conjecture to the level of myth. The entity known as FOURTH disease was NOT a misdiagnosis of SECOND or THIRD disease by back-woods physicians, NOR was it SSSS, NOR is it widely accepted by the medical community that it was merely SSSS in disguise.

Kiss-of-death numbers 9 and 10. Nobody talks about FOURTH disease anymore – that is outside the occasional historical lecture and as obligatory mention during a discussion of the “numbered diseases.”

The Rest Of The Story

If FOURTH disease was real then, has it died off now or is it still around under another name? Who would know? If you don’t know what something looks like, how will you know if you’ve found it – no matter how long you look.

Some Things Change

We now know that there are probably thousands of viruses, many of which cause rashes. There are a slew of what we call “summer rashes” which only cause a rash without much disease and even some stomach “bugs” are known to cause a minimal rash after the vomiting stops.

It’s a lot easier these days to prove that something is caused by a different virus – we have virus testing. The problem is that actually knowing the virus’ name is not only expensive but largely academic because there still isn’t a cure for a virus.

About the only people who actually DO virus testing are the academics; and the last person who would see a patient with FOURTH disease would be an academic – it doesn’t make people sick enough for an academic center.

The CDC’s Mortality and Morbidity Weekly Report (MMWR) in March 2002 published a report entitled “Rashes among schoolchildren” — 14 States, October 4, 2001 – February 27, 2002.” Hundreds of children were caught in an epidemic of rashes which sounded an awful lot like good ‘ol number FOUR. Ten to 25% of school children in the attacked area, generalized rash, a bit itchy, lasting a few hours to a few weeks, mild illness no need for quarantine…

The illnesses were reported, dermatologists saw some and said it “looked a little like FIFTH disease” – but nobody thought to get a viral culture! This is a bit like looking for sasquatch or that Loch Ness fish thing!

Some Things Never Change

Here are but two of the many samples of wisdom in the writings of Dr. Dukes from the time that he was physician at Rugby boarding school in Rugby England.

Clothes and Underwear

“One word more on this subject of clothes. Some boys at our schools, frequently those who come from homes of affluence, might as well be without mothers or sisters, judging from the condition of their clothes, especially of their under-clothing. I know boys are careless in the way they deal with their underclothes, in dressing and undressing; but their under-garments are sent to school from some homes in a state that would disgrace any poor cottager who was short of time to mend, and of means to buy. With some palpable exceptions, I do not think one would be very wide of the mark in judging a boy’s probable character from the state of his underclothes; as this would generally be empathic evidence of whether the boy had been well brought up by his mother, and had known the influence of a mother’s or sister’s love and care.” (Health at School: Considered in Its Mental, Moral, and Physical Aspects (1905), Clement Dukes, )

Arrogant, “Entitled” Personality

“Parents are prone to allow – frequently to encourage – their children to presume upon their noble and ancient birth, and to expect homage on account of ancestry. A long line of noble ancestors (in the true sense of the term) is much to be coveted; but noble birth does not cover worthless personal conduct. Better far, simply to teach children, that each may become, and should strive to be, a noble ancestor to succeeding generations.” (ibid:)

[Public Health Report – https://archive.org/details/healthatschoolc01dukegoog]