Morphea's confounding morphology: the broad definition problems of Eosinophilic fasciitis.
Morphea? Never heard of it. The word sounded ancient and outdated, a relic of a long ago diagnostic past. I rechecked the Autoimmune Association and Global Autoimmune Institute lists of autoimmune disorders. Neither one included Morphea. Some experts consider Eosinophilic fasciitis to be a severe form of Morphea, according to this February 2023 review article* of Eosinophilic fasciitis. If Eosinophilic fasciitis falls under the umbrella definition of Morphea, it becomes clear that there are several broader “umbrella” terms Eosinophilic fasciitis is absorbed by, creating macro definition problems. I wrote about the micro definition problems of Eosinophilic fasciitis in last week’s post here. If you’d like to read more about Eosinophilic fasciitis, please check out its preliminary Diagnosis Description here.
So, what is Morphea?
Morphea (localized scleroderma) is a rare autoimmune connective tissue disease with variable clinical presentations, with an annual incidence of 0.4–2.7 cases per 100,000. Morphea occurs most frequently in children aged 2–14 years, and the disease exhibits a female predominance. Insights into morphea pathogenesis {cause of disease} are often extrapolated from studies of systemic sclerosis {a vital organ-damaging sub-type of Scleroderma} due to their similar skin histopathologic {diseased tissue} features; however, clinically they are two distinct diseases as evidenced by different demographics, clinical features, disease course and prognosis.
(Wenzel et. al, 2021).
So what does Morphea have to do with Eosinophilic fasciitis?
The following is a smorgasbord of diagnoses that all relate to Eosinophilic fasciitis. Please bear with me. Scleroderma (literally meaning “hard skin,” and encompassing all autoimmune disease with the clinical sign of thick, hard skin and/or biopsy-confirmed characteristics of diseased tissue) is split into two categories: Localized scleroderma and Systemic Sclerosis. You can read my work-in-progress Diagnosis Description for Scleroderma here. Morphea is the more child-centric name that falls under the broader category of Localized scleroderma. Localized scleroderma is divided into 5 subtypes where the blurring between the terms Localized scleroderma and Morphea is easily seen:
Plaque morphea
Generalized morphea
Bullous morphea
Linear scleroderma - including subtypes that involve the head and face, linear scleroderma ‘en coup de saber' (LScs) and progressive facial hemiatrophy (PFH) {one-sided arrest of tissue growth that gets worse and worse},
Deep morphea.
(Careta et. al, 2015)
It can be argued that Eosinophilic fasciitis is synonymous with the sub-type of Localized scleroderma known as “Deep morphea.” Why is this tangled hierarchy of conflicting, and ill-defined diagnoses, important to understanding what is known about Eosinophilic fasciitis? Exploring the scientific research for all of these “Diagnoses” is necessary to a full understanding of what is currently known about Eosinophilic fasciitis.
“Morphea” is used to “prevent confusion” by obscuring the connections between Sclerodermal diseases from patients. It’s patronizing and dangerous.
This 2017 review article* on Morphea and Eosinophilic fasciitis outlines how Morphea is used in the United States in place of Localized scleroderma:
However, morphea is the preferred term in the United States (US), because localized scleroderma could lead to unwanted confusion with the term systemic sclerosis, a systemic disorder with a different plethora of clinical and pathological signs and symptoms [1]. For the purpose of this review, we will use the term morphea.
(Mertens et. al, 2017)
I want to briefly address the underlying patronizing message of the above paragraph. What I read into the naming choice of Morphea goes something like this: “if we call Localized scleroderma Morphea, then patients won’t become worried and ask all kinds of questions about systemic sclerosis.” For patients who want to self-educate to self-advocate, I consider this a manipulative use of terminology designed to ease the communication work of the clinician, not to help the patient. It’s also dangerous, because although there are marked clinical differences between Localized scleroderma and Systemic Sclerosis, this 2015 review article* makes clear that the distinctions are by no means clear:
The literature suggests that LoS {Localized scleroderma} is not an exclusively cutaneous {skin} disease.12 There is evidence of involvement of internal organs, association with other connective tissue diseases and exceptional transitional forms for SSc {Systemic sclerosis}, especially in adults with the localized form of the disease.13,14
(Careta et. al, 2015)
Patient awareness of the possibility for overlapping symptoms in all of the conditions falling under the “Scleroderma” (or “hard skin”) umbrella could mean the difference between early and accurate diagnosis that minimizes the chance of irreversible damage. After all, patients live in their body all the time. Clinicians evaluate them only intermittently.
Silos of Specialties Lead to Silos of Diagnoses
Pediatricians saw their patients, and called it Morphea. Dermatologists saw their patients and called it Scleroderma. When Dermatologists determine there’s vital organ disease, they say, “not in my silo,” and send their patients to the silo of Rheumatology, who call it Systemic sclerosis. In the absence of scientific research on the shared, or disparate, disease-causing mechanisms (genetic, environmental and immune) of these diagnoses, across medical specialties, it’s impossible to know exactly where to place Eosinophilic fasciitis in relation to Scleroderma, Systemic sclerosis, Localized scleroderma, and Morphea. For now, the shared clinical sign of thick, hardened skin and/or characteristic fascia biopsy findings, link these conditions in their definitions and their differentiations.
Next Week…
A post about differentiating Eosinophilic fasciitis from other types of Sclerodermas. For those who are new to AutoimmuneDx, I am currently writing posts based on reader-requests for more information and analysis on particular autoimmune diagnoses. If you would like me to take a closer look at a particular diagnosis, please leave a comment below. If you don’t feel comfortable commenting publicly, email me at autoimmunedx@gmail.com. If you would like me to clarify a post, a concept, a word, or anything in-between, please don’t hesitate to leave a comment or send an email.
*A Note About Review Articles
A review article is a compilation and interpretation of the scientific research to date. It is useful for getting a broad overview of current topics for a given diagnosis, and relevant citations to explore. When I look for review articles, I try to find the most recent, with as many co-authors as possible, to indicate at least some professional consensus. It is more rigorous to go to the source material directly and evaluate study conclusions yourself, but for orientation to a given diagnosis, review articles are invaluable. It’s also useful to read what physicians are reading and writing (review articles!) to stay up-to-date in their particular disciplines.
References
Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015 Jan-Feb;90(1):62-73. doi: 10.1590/abd1806-4841.20152890. PMID: 25672301; PMCID: PMC4323700.
Mazilu D, Boltașiu Tătaru LA, Mardale DA, Bijă MS, Ismail S, Zanfir V, Negoi F, Balanescu AR. Eosinophilic Fasciitis: Current and Remaining Challenges. Int J Mol Sci. 2023 Jan 19;24(3):1982. doi: 10.3390/ijms24031982. PMID: 36768300; PMCID: PMC9916848.
Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong EMGJ. Morphea and Eosinophilic Fasciitis: An Update. Am J Clin Dermatol. 2017 Aug;18(4):491-512. doi: 10.1007/s40257-017-0269-x. PMID: 28303481; PMCID: PMC5506513.
Wenzel D, Haddadi NS, Afshari K, Richmond JM, Rashighi M. Upcoming treatments for morphea. Immun Inflamm Dis. 2021 Dec;9(4):1101-1145. doi: 10.1002/iid3.475. Epub 2021 Jul 17. PMID: 34272836; PMCID: PMC8589364.