In my ongoing quest for ichthyosis in history, I came across old dermatology texts from 1874 and 1895, co-written by Doctors Ferdinand Hebra and Moriz Kaposi. (Today, Kaposi might be best-known for first describing a type of skin cancer modern medicine calls “Kaposi’s sarcoma”.) These old texts were really fascinating to me, in a rubbernecking, OMG! sort of way. I’m already the sort of person that likes to visit the Civil War-era surgery museums filled with exhibits of rusty pliers, snake-oil medicines, battlefield medicine exhibits and giant hairballs (seriously), so this puppy was right up my alley.
Did you know that ichthyosis was first described as early as 1025 A.D.? A Persian scholar named Avicenna from modern-day Uzbekistan wrote a 14-volume tome that described over 2000 diseases and disorders, and that tome, the Canon of Medicine, was the main medical book used to train doctors over the next 700 years. (Can you even imagine? 700! Nowadays, aren’t things considered outdated if they’re more than a couple of years old?) Avicenna used the name “albarras nigra” to describe ichthyosis.
Avicenna’s original text was in Arabic, but the Latin translation is quoted in searchable editions online, and it is also quoted in Hebra and Kaposi’s text. So the source material from 1,000 years ago says, “Est scabiositas accidens cuti aspera vehemens, et facit squamas sicuti sunt piscium.” Or (forgive our rusty Latin): “It is a type of scabies occurring randomly and causing severe roughness to the skin, making it appear as though one were looking at a fish.”
A few other people mentioned ichthyosis here and there in medical literature, but it was never called ichthyosis. Mostly, it was still considered a form of leprosy, like “leprae leontiasis.” In 1710 a guy named Edward Lambert was born in Ireland and grew up to have 2 kids affected by what we would now call ichthyosis hystrix — a dominant sort of ichthyosis where the skin forms giant spikes and thick scales like an armadillo. Lambert and his sons traveled around a bit as sort of a traveling circus exhibit to earn money and made their way to Germany. In 1755, poor Edward was diagnosed with “Stuchelschweinmench,” or “porcupine man”. Late in his life, Lambert met another doctor that wrote about him and decided to rename the disorder “Krustenmann” — literally, “crusty man”. Uh…yay?
By the late 1700s, the Germans had settled on the name “Fischhschuppenkrankheit”, which literally means “fish skin disorder” and pretty much meant what we now call ichthyosis vulgaris (IV). Since most medical stuff uses Greek or Latin, they eventually abandoned the German; in 1808 Robert Willan wrote a detailed description of all known types of skin lesions, which caused the definitive switch from leprae to ichthyosis, ikhthys (Greek for fish) and osis (Latin for condition).
In the 1874 book, I learned that there was still doubt about whether ichthyosis vulgaris was inherited or not. Remember, at the time Hebra and Kaposi’s text was first published, Gregor Mendel had finished his pea-plant experiments only 10 years previously. Because the cause of the doubt was both the severity of the problem and the assertion that ichthyosis did not develop for up to 2 years after birth, the discussions about “ichthyosis” and “ichthyosis simplex” (or other related conditions) in those old texts are almost certainly about the common forms of ichthyosis, such as ichthyosis vulgaris or X-linked ichthyosis.
For the more severe forms of ichthyosis, conditions we now know as epidermolytic ichthyosis or autosomal recessive congenital ichthyosis (ARCI), at least some of those types of ichthyosis were known to the authors of these older texts. In another dermatology textbook from 1866 named, “A Practical Treatise on Diseases of the Skin“, Dr. John Moore Neligan wondered whether ichthyosis was a type of leprosy or a psoriasis or a scabies, or if it was in a class by itself. Neligan described several examples well enough to recognize them today. He also wrote about some more severe forms appearing out of the blue from families with no history of skin birth defects.
Hebra and Kaposi described “Ichthyosis congenita“, a condition they called a “rare affection…in which the skin of the newly-born child is of a brownish-red colour, sward-like, fissured, and, owing to the fissures, its surface appears mapped out into large plates, and is in a condition like that of the skin of a roasted apple, or of a roasted sucking-pig…or resembles the appearance produced by scalding the skin with a moderately hot fluid.” The reference to “large plates” certainly sounds like harlequin ichthyosis to us! (We are also amused and somewhat horrified, from a modern patient’s perspective, that this colorful description found its way into the leading medical text of its time. It says much about the attitudes of the time; Rachel remembers hearing about contemporaneous doctors in Paris informing terminally ill patients that they were “looking forward” to being able to conduct autopsies on them…)
While Hebra and Kaposi wrote back then that “the children affected lived only for a few days”, they also described “a case in which a child affected with this deformity was not only kept alive by careful nursing and appropriate treatment, but is still at the present time, after several years, perfectly well.” There was doubt whether collodion babies were even affected with ichthyosis. One of the arguments of the day was whether the lamellar form was “ichthyosis sebacea” or “cutis testacen,” and whether it was related to ichthyosis simplex. And as best as I can tell, epidermolytic ichthyosis isn’t even described in these texts.
But in general, Hebra and Kaposi’s experience with ichthyosis is that the children “show no abnormality of their skin whatever, at birth…[and] it is only at a more advanced period, at about the age of 2 years, that the first symptoms of ichthyosis usually become visible.” So based on that alone, it seems clear to us that the typical ichthyosis described by Hebra and Kaposi back in 1874 were what we now call ichthyosis vulgaris and X-linked ichthyosis.
We’ve come a long way from “porcupine man” and “skin of a roasted apple,” and I am very glad of that!
This post is part of a series. Here are links to the others: