When last I blogged, we examined Lynch's depiction of Merrick's fulfillment for the general criteria of Proteus syndrome, which included a mosaic distribution of lesions, sporadic occurrence, and a progressive course the condition. Now, let's take some time to look at Merrick's relationship to the specific criteria for PS.
THERE'S A LOT TO CHOOSE FROM...
Here is a close up of half of the diagnostic chart we used last time.
Now, in The Elephant Man, Lynch has to show us that Merrick claims either the single condition from "Category A" (Cerebriform connective tissue nevus,) or two conditions from "Category B" (Linear epidermal nevus, asymmetric disproportionate overgrowth, specific tumors before the second decade), or three from "Category C" (Disregulated adipose tissue, vascular malformations, lungcysts, facial phenotype.)
How can you do that in a movie? Not very easily. But before we glaze over from the long words, let’s look at Category A - cerebriform connective tissue nevus. If Merrick is qualified through this single category, then Lynch's work is over (ha!)
NEVI, NEVI, AND MORE NEVI
First of all a “nevus” is usually a congenital(1) pigmented skin growth like a mole or a birthmark, or it can be a skin growth with a lot of blood vessels in it, or any one of a bunch of benign skin growths out there. Nevi can also be any size. Moles are nevi. Small nevi are not usually a big deal. Large nevi can be very disfiguring. Some types of nevi can have systemic effects(2), but most do not.
In relation to Proteus syndrome, a connective tissue nevus is a skin growth made of hyper-active connective tissue cells (as opposed to skin cells), and “cerebriform” means that it has contours that make it look like the surface of a brain.
Look at a picture of this foot with CCTN,
and look at the film version of Merrick’s back. The texture is very close and very brain-like towards the left side of Merrick's back. These make up guys are really good.
Technically, we could stop here with the diagnosis, since Merrick only has to have the single condition in category A. If we look at Category B, we see another type of nevus called a linear epidermal nevus, which (Yes, you guessed it!) are a series of nevi distributed in a linear pattern.
The two conditions from Category B that would be easy to depict on film are the linear epidermal nevus, which Merrick doesn't display, and the asymmetric, disproportional over growth of the limbs and/or head, which he does. Hypertosis of the skull means increase bone growth. We will look at this along with megaspondylodysplasia, next time.
THE TUMOR THING
As for tumor formation from Category B, parotid monomorphic adenoma is a tumor of the parotid gland, which is a salivary gland. Merrick's face is so deformed with bony hypertrophy and connective tissue overgrowth, that it's entirely possible he had a tumor inside his cheek. On the other hand, we can be pretty darn sure he didn't have ovaries, so those tumors are out.
Just to linger on the tumor thing for a bit longer, surgery and medicine was not very advanced in the late 1800s. With everything else that Merrick had going on it was unlikely that internal tumors would be addressed unless they were extreme and/or treatable. Tumors were usually only diagnosed and treated when they were big enough to cause pain and threaten one's life. Antiseptic practices were still controversial and not only did one have to survive potential sepsis post-surgery(3), but you had to survive surgery itself, which wasn't always easy. Although Merrick's "tusk-like" tooth is mentioned in the film, it would be hard to credibly bring up a parotid tumor.
Next time, I'd like to continue to look at some of the criteria in categories B and C for their expression in Merrick's body in the film and in real life, as well as some of the symptoms Merrick might have felt as a result of this tissue growth.
1. Congenital: Disease or condition present at birth or that manifests within the first five years of life. Congenital conditions may be hereditary, caused by disease, birth trauma, poisoning in utero, etc. Some people will also use the term congenital to describe any genetic condition, even if it manifests in adulthood. As far as I know, Congenital Liars have not been recognized as a medically diagnosable group, nor are there studies out there looking for genetic markers that may pre-dispose someone to being a congenital liar.
2. Other organs or systems in the body may be affected.
3. Once in later posts I'll discuss the surgery scene at the opening of the film, and antiseptic practices of the day.
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