Leprosy In The Medical Casebook

 

This Week

I said last week that we would next have a look at a short chapter in leprosy … but the chapter is actually fairly long, as I found lots of interesting information in the Victorian textbooks on what was an important [and scary] medical condition at the time.

 

Leprosy

Leprosy + Sherlock

Leprosy plays a key role in “The Adventure Of The Blanched Soldier”. The story is narrated by Holmes, as Watson has “deserted him for a wife.”  Holmes is engaged by an ex-soldier, James M Dodd, who is trying to make contact with his close friend Godfrey Emsworth. Dodd visits the family house, where he is prevented from seeing Godfrey by his father, Colonel Emsworth.

That night, whilst looking out of his bedroom window.

“He was outside the window, Mr Holmes, with his face pressed against the glass. I have told you that I looked out at the night. When I did so I left the curtains partly open. His figure was framed in this gap. The window came down to the ground and I could see the whole length of it, but it was his face which held my gaze. He was deadly pale – never have I seen a man so white. I reckon ghosts may look like that; but his eyes met mine, and they were the eyes of a living man. He sprang back when he saw that I was looking at him, and he vanished into the darkness.”

Dodd pursues Godfrey, but the colonel intervenes, and he learns no more. Holmes develops three possible theories, then discounts two of them as unlikely [that Godfrey has committed a crime, and that Godfrey is insane]. Which leaves his third theory as the only possible outcome – that Godfrey has a disease, causing disfigurement, and requiring segregation.  

Eventually Holmes forces the Colonel’s hand, and they are taken to meet Godfrey.

“Don’t touch me, Jimmie. Keep your distance. Yes, you may well stare! I don’t quite look the smart Lance-Corporal Emsworth, of B Squadron, do I?”

His appearance was certainly extraordinary. One could see that he had indeed been a handsome man with clear-cut features sunburned by an African sun, but mottled in patches over this darker surface were curious white patches which had bleached his skin.

Fleeing a fight in the Boer War, in which he is wounded, Godfrey seeks shelter in a house in the dark. In the morning he realises he has made a terrible mistake.

In front of me was standing a small, dwarf-like man with a huge, bulbous head, who was jabbering excitedly in Dutch, waving two horrible hands which looked to me like brown sponges. Behind him stood a group of people who seemed to be intensely amused by the situation, but a chill came over me as I looked at them. Not one of them was a normal human being. Every one was twisted or swollen or disfigured in some strange way. The laughter of these strange monstrosities was a dreadful thing to hear.

An elderly Dutch doctor intervenes.

“How in the world did you come here?” he asked in amazement. “Wait a bit! I see that you are tired out and that wounded shoulder of yours wants looking after. I am a doctor, and I’ll soon have you tied up. But, man alive! you are in far greater danger here than ever you were on the battlefield. You are in the Leper Hospital, and you have slept in a leper’s bed.”

The Colonel explains how Sherlock persuaded him to reveal all.

Colonel Emsworth pointed to me.

“This is the gentleman who forced my hand.” He unfolded the scrap of paper on which I had written the word ‘Leprosy’. It seemed to me that if he knew so much as that it was safer that he should know all.”

Holmes explains how he had arrived at this diagnosis.

There remained the third possibility, into which, rare and unlikely as it was, everything seemed to fit. Leprosy is not uncommon in South Africa. By some extraordinary chance this youth might have contracted it. His people would be placed in a very dreadful position, since they would desire to save him from segregation. Great secrecy would be needed to prevent rumours from getting about and subsequent interference by the authorities. A devoted medical man, if sufficiently paid, would easily be found to take charge of the sufferer. There would be no reason why the latter should not be allowed freedom after dark. Bleaching of the skin is a common result of the disease.

Holmes, however, opts for a second opinion, and we shall return to the story in a few chapters … 

Though published in 1926, the story is set in 1903, at the conclusion of the Boer War. Conan Doyle spent five months in South Africa in 1900, serving as a volunteer medic. One wonders if he encountered any leprosy cases, or based “The Adventure Of The Blanched Soldier” on textbook learning. I suspect the latter, as his description of the Leper Hospital seems more sensationalist than factual. 

Leprosy makes an appearance in two other stories. In “The Yellow Face”, which we will visit in more detail during our Yellow Fever chapter, leprosy is one of Sherlock’s theories as to whom Effie Munro is hiding away in a remote cottage [so a similar plot to “The Adventure Of The Blanched Soldier”].

“You have a theory?”

“Yes, a provisional one. But I shall be surprised if it does not turn out to be correct. This woman’s first husband is in that cottage.”

“Why do you think so?”

“How else can we explain her frenzied anxiety that her second one should not enter it? The facts, as I read them, are something like this: This woman was married in America. Her husband developed some hateful qualities; or shall we say that he contracted some loathsome disease, and became a leper or an imbecile? She flies from him at last, returns to England, changes her name, and starts her life, as she thinks, afresh.“ 

Finally we get two references in “The Adventure Of The Illustrious Client”, which we have just encountered in the preceding Scurvy chapter.

The first quote follows on directly from that in the previous chapter. Porky Shinwell introduces Holmes and Watson to Miss Kitty Winter, the rejected mistress of Baron Gruner.

It seems that he dived down into what was peculiarly his kingdom, and beside him on the settee was a brand which he had brought up in the shape of a slim, flame-like young woman with a pale, intense face, youthful, and yet so worn with sin and sorrow that one read the terrible years which had left their leprous mark on her.

“This is Miss Kitty Winter, said Shinwell Johnson, waving his fat hand as an introduction.”

Here, the term “leprous” is being used metaphorically, in the sense of Kitty being disfigured by the awful life she has had to lead.

Kitty then joins Holmes and Watson in a visit to Violet de Merville, in an attempt to persuade her of the evil nature of her fiancée.

She knew what we had come for, of course – that villain had lost no time in poisoning her mind against us. Miss Winter’s advent rather amazed her I think, but she waved us into our respective chairs like a reverend abbess receiving two rather leprous mendicants. If your head is inclined to swell, my dear Watson, take a course of Miss Violet De Merville.” 

It would appear that Holmes and Watson were offered chairs, but that Kitty Winter was seen as below even having leprosy, and was made to stand.

We will pick up the exciting story again in our Erysipelas chapter.

 

Leprosy In Victorian Times

Elephantiasis Graecorum is a chronic constitutional disease characterised by structural changes in the skin, mucous membranes and nerves, and producing great disfigurement of the features and deformity of the extremities. [12]

This disease is very widely distributed and found in every quarter of the globe, but is far more common in tropical than in temperate climates. It was very prevalent in Europe during the Middle Ages, but since that time it has almost died out, and is now only found in circumscribed districts, of which the most extensive is the west coast of Norway. [12]

Leprosy has gone under various names historically. The Romans coined the term Elephantiasis Graecorum, I assume looking to blame the Greeks for the unpleasant disease. Similarly they had a disease called Elephantiasis Arabum, blamed on the Arabs [now called lymphatic filiariasis]. Leprosy has also previously been called Hansen’s disease.

My very limited encounters with the disease have involved, I’m afraid, the key doctor skill of blagging. As a medical student, I did an elective posting at the Gurkha Military Headquarters in Dharan, Nepal. I was left running the medical clinic one day. An ex-Gurkha had come all the way from Burma [now Myanmar] to have his leprosy treatment reviewed. Luckily, he was doing well, and I could just provide more of the treatment. I also have a current role as a tutor for a postgraduate course in dermatology at Cardiff University. I once had to mark a case history on leprosy, written by a student who was the head of an African leprosy charity. He knew way more than I did … and scored very high marks. 

It has been ascertained beyond all doubt that the disease is hereditary, and this is probably the chief cause of its continuing to exist in such countries as Norway and Iceland, where intermarriages amongst the afflicted families are common. Certain conditions of climate, soil and food appear also to have some influence on its development. [12]

The question whether leprosy is contagious or not was answered by the College of Physicians’ report of 1867, and that of the Hawaiian Government in 1886, in the negative, while the College Of Physicians now admits that the question is quite an open one. There is, indeed, much evidence of its being inoculable even by vaccination, while coitus, prolonged contact, and even breathing in the same atmosphere for a long period, seem to have produced it in some instances. The invariable presence of bacilli in tissues, and the fact the prevalence of leprosy in Norway has been diminished by 50 per cent in twenty years by strict segregation, are facts which are also in favour of the contagious theory. [13] 

Modern research is strongly in favour of the disease being one of constitutional origin, closely analogous to syphilis, in which special bacilli, either directly or indirectly, by their presence set up inflammatory changes in the tissues, to which many of the lesions are due. [13]

The bacilli were discovered by Hansen, in 1874. They are straight or very slightly curved rods, half to three quarters of the diameter of a red corpuscle in length [4 to 6 mm or 1/5000 inch]. [13]

It is fascinating to see the Victorian medics wrangling with theories of disease. The aetiological information in the earlier Liveing text of 1880 [which is very categorical] has been superseded in the later Radcliffe Crocker text [1888] by a more considered approach to new disease theories.

We now know leprosy is a bacterial disease, caused by Mycobacterium leprae [we will meet other Mycobacterium bacteria in our Tuberculosis chapter]. Interestingly it is still not completely certain how leprosy can be spread, though droplet spread via breath is the main disease vector.

I was intrigued that a metric measuring system was given for the bacteria size. I assume this was used in some scientific papers [the metric system actually dates back to the 17th century]. Though the use of millimetres seems completely wrong – it is out by a factor of 1000 – yet I guess mm could mean micrometre, which would be correct, and an amazingly early use of the term.

The invasion of the disease is usually slow and insidious; often years elapse before any very characteristic symptoms appear. In exceptional cases, however, the onset of the disease is acute, and the symptoms develop with great rapidity. [9]

The early symptoms of the malady consist of general constitutional disturbance, debility, mental depression, loss of appetite, chilliness, and slight recurrent febrile attacks. [12]

As a further and later change, very characteristic tubercles develop in the skin, especially on the face and hands; these swellings are tender on pressure, and they produce much thickening of the tissues and consequent alteration in the features. The skin of the forehead becomes thickened and tuberculated, its furrows deepened, and its prominences exaggerated; this is especially the case on and over the eyebrows, and gives a peculiar heavy, morose expression to the countenance; the hair of the eyebrows is quickly lost, the nose becomes tumid with nodules and tubercles; the cheeks are irregularly thickened, the lips hard, swollen, and sometimes everted; the chin is nodulated, and the ears, greatly enlarged, stand out stiffly from the side of the head. The whole appearance is hideous and revolting. One peculiar effect of these changes is to make young people look middle-aged. [12]

In other cases great atrophy and stiffening of the fingers occurs, so that they assume the appearance of shrunken immovable claws. Sometimes the parts fall off without giving any pain. [12]

In males the testicles atrophy, the breasts enlarge, and sexual power is lost; women become sterile, the voice gets croaking from tubercles in the larynx, there is snuffling from thickening of the nasal mucous membrane, a kind of pannus may ensue on the conjunctiva and cornea and interstitial keratitis, and corneal tubercles may lead to blindness. [13]

The disease is almost invariably fatal, and even though existence is prolonged for many years, it is at best a miserable one. [13]

Leprosy sounds a horrendous disease, and one we should be extremely grateful that we don’t have to fear. However, it remains an important condition in developing countries – the World Health Organisation tells me that there were just over 200,000 new cases in 2019.

From the comparative rarity of true leprosy in this country it is very difficult to obtain a sufficient number of cases on which to base our conclusions as to the effect of treatment, and therefore the positive results of any experiments in the use of drugs must be received with caution. [12]

Treatment. This, unfortunately, can only be palliative or preventative, the number of so-called specifics bearing testimony to the incurability of the disease. Of the many recommended only two have stood the test of long experience – Chaulmoogra oil from gynocardia odorata, and Gurjun oil from dipterocarpus laevis. These oils are taken internally and rubbed in externally; both are very nauseous, and are best given in emulsions or pearls, beginning with small doses. [13]

As preventive measures, strict segregation is the only effective plan, and it is probable that the disease was stamped out of England and the greater part of Europe by these means, and great diminution in the number of lepers has ensued in Norway since its adoption. Those who dress the sores of lepers should be very careful if they have scratches or abrasions, and not neglect carbolic-acid ablutions afterwards. [13]

Chaulmoogra and Gurjun oils are both old Indian Ayurvedic treatments, becoming widely used leprosy treatments in the UK in the 19th century [probably brought home by Indian settlers who contracted leprosy]. Gurjun oil is much the same as Balsam oil.

Segregation was of course carried out via leper colonies, which were widely used through the Middle Ages, often run by monastic orders. As grim as they undoubtedly were, they were potentially an effective method of public health control of leprosy, being effectively an ancient equivalent of COVID isolation.    

These days leprosy is a curable disease, using a combination of 3 drugs [the MDT regime – dapsone, rifampicin and clofazimine]. It is an interesting example of the WHO and a drug company working very effectively together, as Novartis provide the drugs free to anyone in the world with leprosy. Leprosy is a disease that could theoretically be eliminated globally, assuming you can get the drugs to those with early infections.

 

Next Week

The above chapter references an ensuing chapter on Erysipelas, so let’s visit that topic next. Erysipelas is a rare infection these days, generally easily treated with antibiotics. But, as you might expect, in Victorian times it was much more serious – such that it is used as a reason Sherlock Holmes is on death’s door in one of the stories. And the following week we'll visit the conclusion of the "Adventure Of The Blanched Soldier".

 

Sherlock Holmes And The Adventure Of The Ordered Occupations

Another story to accompany publication of The Medical Casebook Of Sherlock Holmes And Dr John Watson. 

Dr Watson attends a hansom cab accident on Kensington High Street, in which a fellow general practitioner is seriously injured. Acting as a locum for his colleague, Watson is called to a mysterious death. Sherlock Holmes is asked for help, and discovers another similar death. Unravelling the mystery makes full use of Watson’s medical knowledge, but can our heroes prevent further deaths?

Available from the Amazon Kindle Store.

 

Buying The Book

The Medical Casebook of Sherlock Holmes and Dr John Watson is available from all good bookstores including Amazon USA, Barnes and Noble, Amazon UK and additional formats like Kindle.