Ichthyosis In The Medical Casebook

“Godfrey Emsworth appears at the window of his friend’s bedroom.” © Alex Holt

This Week

We are going to return to “The Adventure Of The Blanched Soldier” and find out the actual diagnosis for Godfrey’s Emsworth’s skin disease. This page covers a fairly obscure disease – but hopefully one that is still of interest.

News

The book is now published and is fully available.

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.

 

The Illustrator

Our page is headed with another marvellous original illustration by Alex Holt.

Alex Holt is an artist specialising in original ink illustrations. He has a special interest in Comic Art, single image illustrations and covers. Alex is a student of the Edinburgh Atelier of Fine Art. He works in private commissions and commercial projects. More of his work can be found in Instagram @alexholtart.

 

Ichthyosis

Ichthyosis + Sherlock 

A few chapters back, in “The Adventure Of The Blanched Soldier”, we left Godfrey Emsworth being hidden away by his family, having been thought to have contracted leprosy during the Boer War. Having forced the Emsworth family into revealing their story, Holmes takes the role of an assertive patient representative pushing for a second opinion.

“I understand that only Mr. Kent has seen the patient. May I ask, sir, if you are an authority on such complaints, which are, I understand, tropical or semi-tropical in their nature?”

“I have the ordinary knowledge of the educated medical man,” he observed with some stiffness.

“I have no doubt, sir, that you are fully competent, but I am sure that you will agree that in such a case a second opinion is valuable. You have avoided this, I understand, for fear that pressure should be put upon you to segregate the patient.”

“That is so,” said Colonel Emsworth.

“I foresaw this situation,” I explained, “and I have brought with me a friend whose discretion may absolutely be trusted. I was able once to do him a professional service, and he is ready to advise as a friend rather than as a specialist. His name is Sir James Saunders.”

The prospect of an interview with Lord Roberts would not have excited greater wonder and pleasure in a raw subaltern than was now reflected upon the face of Mr. Kent.

“I shall indeed be proud,” he murmured.

“Then I will ask Sir James to step this way. He is at present in the carriage outside the door.”

Whilst Godfrey gets his second opinion, Holmes has time to explain his own diagnostic method to his father.

I was finishing this little analysis of the case when the door was opened and the austere figure of the great dermatologist was ushered in. But for once his sphinx-like features had relaxed and there was a warm humanity in his eyes. He strode up to Colonel Emsworth and shook him by the hand.

“It is often my lot to bring ill-tidings and seldom good,” said he. “This occasion is the more welcome. It is not leprosy.”

“What?”

“A well-marked case of pseudo-leprosy or ichthyosis, a scale-like affection of the skin, unsightly, obstinate, but possibly curable, and certainly non-infective. Yes, Mr. Holmes, the coincidence is a remarkable one. But is it coincidence? Are there not subtle forces at work of which we know little? Are we assured that the apprehension from which this young man has no doubt suffered terribly since his exposure to its contagion may not produce a physical effect which simulates that which it fears?”

I have worked as a specialist dermatologist, as well as being a GP, and this is one of my favourite Sherlock cases. It is not often that the key plot point in a work of fiction is a dermatology misdiagnosis.  

 

Ichthyosis In Victorian Times

The name is derived from the resemblance of the skin to the skin of a fish. The disease seems to consist of an increased thickness and altered condition of the epidermis, and is sometimes accompanied by hypertrophy of the papillae of the cutis. It is often hereditary, and in some cases, congenital. [10]

Ichthyosis is a congenital hypertrophic disease of the skin, characterised by increased growth of the papillary layer with thickening of the true skin and the production of masses of epidermic scales. Some doubt whether ichthyosis is always a congenital disease. The characteristic appearance of the skin is sometimes not developed until some years after birth. [18]

“Congenital” and “hereditary” are overlapping terms. A congenital disease is one that is present at birth – which may be due a genetic defect, or due to environmental factors. A hereditary disease is inherited from your genes, and may be present at birth, or may only manifest later in life.

Ichthyosis is a fairly rare skin condition, and quite a complex one. The term means that a patient has an abnormality in the process of skin cornification, which is the process whereby living skin cells [keratinocytes] die and form a protective layer of dead skin cells [corneocytes]. We need to have some corneocytes, but in ichthyosis you get excessive numbers, which manifests as markedly scaly skin.

There are a range of genetic causes, but it can also develop later in life for assorted reasons. It can occasionally be a skin manifestation of disease elsewhere, such as lymphoma or HIV infection.

There is, however, no evidence that it is a psychosomatic disease, which is the theory that Sir James Saunders puts forward for how Godfrey Emsworth developed the condition. 

As an additional aside, this is our first reference to the work of Dr Malcom Morris, a London dermatologist. He was a close friend of Arthur Conan Doyle, and, according to Conan Doyle’s autobiography [19], was the main influence on him leaving general practice.

In ichthyosis or fish-skin-like-disease there is an incrustation of blackish colour on the fronts of the knees or in other parts of the body. The incrustation is composed of epithelial scales and sebum. [5]

There are many degrees of ichthyosis ranging from a mere roughness of the skin to a condition resembling that of the skin of the shark. In a case of average severity the growth of the papillary layer is greatly increased, and the whole skin thickened. The natural furrows of the skin are, as the result of this growth, much deepened, and the surface is mapped out onto polygonal tracts, presenting an appearance similar to a crocodile. There is a complete absence of perspiration from the parts attacked with the disease, but the unaffected regions, such as the head, usually perspire freely. [18]

Ichthyosis is a very intractable disease, but is never fatal. It can be somewhat benefited by remedies, but only temporarily. [18]

Anointing the skin with glycerine or vaseline, and the frequent use of bran baths is the treatment. [5]

Any local application likely to soften and remove the accumulated epidermis is the proper treatment, and for this purpose alkaline lotions, and soaps of various kinds, the one recommended by Hebra being composed of iodide of sulphur, oil, glycerine, have been tried. Absolute cleanliness is most essential, and the constant and prolonged soaking of the body in water will often lead to a diminution of the ichthyotic condition. Various internal remedies have been tried, but with no benefit. [18]

Ichthyosis is a rare example in this book, as modern treatment of the condition is essentially the same as the Victorian management. We just use copious amounts of emollients, and nothing else has been discovered that significantly improves the disease. Even the constituents of the Victorian moisturisers are similar to the ones we use today.

Given that Vaseline is a tradename, I was fascinated to see it existed in 1879, when the Morris dermatology text was written. Indeed, the first patent was taken out in 1872, by an American chemist called Robert Chesebrough. It was manufactured by the Chesebrough Manufacturing Company until 1987, when Unilever purchased the firm.

 

Next Week 

Now the book is out, I think the blog has almost run its course. I am starting to run out of interesting chapters to post about. We’ll run the blog for 2 more weeks, which will take us to 20 posts.

Next week we’ll take a look at cataracts, which is an interesting area as Sir Arthur Conan Doyle spent a brief spell as an ophthalmic specialist. Then we’ll finish with tuberculosis the following week.