Losing Face: The
Personal and Social Impact of Scleroderma-Related Facial Changes,
Part 1
by Sharon Wood,
Psy.D. (originally published in "Scleroderma Voice,"
2002 #2)
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Perhaps nothing is so eloquent and significant as the
human face. Looking at one another is our most basic form
of conversation, and wherever people meet in a primary or
face-to-face relationship it is the face, which is generally
the center of attention. It is the source of vocal communication,
the expressor of emotions, and the revealer of personality
traits. The face is the person him/herself. (Macgregor, 1951,
p. 630)
If, as the above
author contends, the human face is deeply and profoundly significant,
what happens when an individual "loses face," both
literally and figuratively? What happens when an individual,
as the result of an illness, accident, or injury, is confronted
with an unwanted and unanticipated change in facial appearance?
What happens when there is very little, if anything, an individual
can do to remedy this change?
These are certainly some of the questions, even if unspoken,
that many individuals with scleroderma-related facial changes
live with on a daily basis as they move through their personal
and social worlds, catching a glimpse of themselves in a mirror
or store window, or in the reaction of others around them,
be they significant or mere strangers. These are questions
that may never have a satisfactory resolution; yet they are
questions whose answers will ultimately define the individual
who has been asked.
This article is the first in a series of several articles
to be published in successive issues of "Scleroderma
Voice" magazine. The articles are based on the author's
recently published dissertation, entitled, "Losing Face:
The Effects of Scleroderma-Related Facial Changes on the Relationship
with Self and Others."
This first article will focus on
four main areas of discussion:
- the physical and functional changes that occur as a result
of scleroderma-related facial changes;
- the author's personal experience with scleroderma-related
facial changes;
- demographics of the participants who took part in the
author's study; and
- participants' personal and interpersonal experiences with
the physical and functional changes that accompany scleroderma-related
facial changes.
The second article will focus primarily on the results of
this dissertation study, particularly the intra- and interpersonal
issues involved in what the author has identified as "the
process of losing face."
The final article will offer an interpretation of the results,
as well as recommendations for how individuals with scleroderma-related
facial changes and those who are an integral part of their
lives, including loved ones and health care professionals,
can relate and work effectively with each other regarding
these changes.
How Likely Are Facial Changes in
Scleroderma?
Scleroderma-related facial changes are a very real possibility
for the vast majority of individuals with systemic (diffuse
and limited) scleroderma and individuals with localized linear
scleroderma en coup de sabre. Not only are facial changes
a very real possibility, they may be one of the many potentially
frightening and disheartening experiences that accompany this
illness.
Currently, there is no available data regarding the precise
number of patients who develop facial changes as a result
of contracting scleroderma. However, one medical researcher
speculates that essentially 100% of the patients with systemic
forms of scleroderma develop this condition as do approximately
20% of those with localized scleroderma (D. E. Furst, Personal
Communication, February 5, 2000).
Incidentally, this author uses the term "facial changes"
as opposed to "facial change" in describing the
effects of this illness on facial appearance because of the
progressive rather than static nature of these changes.
Systemic Scleroderma-Related Facial
Changes
As is true for scleroderma in general, facial changes vary
in their course and effects from patient to patient and over
time in any given individual. In the majority of cases, however,
facial appearance for individuals with systemic and linear
scleroderma en coup de sabre is altered slowly over
time, with changes occurring over several months to a number
of years.
In systemic scleroderma there are three recognized phases
of skin thickening during the facial change process: the edematous,
indurative, and atrophic phases (Clements &
Medsger, 1996). These phases generally follow one another
as the illness progresses.
In the edematous phase, pitting or nonpitting edema
(swelling) may occur in the face and last indefinitely, but
the swelling is usually painless.
During the indurative phase, the edema is gradually
replaced by a markedly thickened dermis and an increasingly
thinned epidermal layer, leading to the loss of skin creases
and a "choking out" of hair follicles, sweat glands,
and sebaceous glands. The latter process is caused by collagenous
deposition in the upper dermis.
In the diffuse form of scleroderma, this process occurs over
several months or years, whereas in the limited form of scleroderma
(known as the CREST syndrome), this phase may occur slowly
over several years, and up to 20 years.
In terms of facial changes, this
entire process results in a "characteristic 'pinched
nose' or 'mauskopf' appearance, sometimes interpreted as an
'expressionless' facies due to reduced mobility of the eyelids,
cheeks, nose, and mouth during ordinary conversation"
(Clements & Medsger, 1996, p. 390).
In the atrophic phase, usually
occurring after several years, the thickened dermis softens
and either reverts to normal thickness or actually becomes
thinner than normal.
A "binding down" or "tethering"
occurs when the dermis becomes more firmly attached to underlying
subcutaneous fat. In this late stage, telangiectasias (clusters
of dilated and tortuous capillaries and venules) increase
in number and tend to be the dominant visual feature, appearing
as a single red spot or in clusters on both cheeks and over
the nose (Clements & Medsger, 1996). The latter shape
resembles a butterfly and is known as the "butterfly-look."
The vast majority
of functional problems that result from systemic scleroderma-related
facial changes are due to the effects of microstomia or "small
mouth."
In addition to
physical pain, the effects of microstomia include:
- tightly pursed lips that lose their fullness;
- peri oral subcutaneous fibrosis; and
- temporomandibular joint (TMJ) involvement.
These effects all
contribute to reduced oral aperture and radial furrowing around
the mouth. Lip retraction, which accentuates the prominence
of the central incisor teeth, also makes normal mouth closure
impossible (Clements & Medsger, 1996).
Microstomia greatly
interferes with several critical functions including eating,
speaking, and proper prophylactic oral hygiene. Many scleroderma
patients also suffer from a constriction in the movement of
the tongue.
If present, secondary
Sjögren's (sicca) syndrome, which interferes with the
normal production of saliva, may also offer an additional
threat to dentition, that is, the appearance and arrangement
of the teeth (Clements & Medsger, 1996).
"Secondary"
Sjögren's syndrome, as opposed to "primary,"
refers to the two forms of this disorder. Secondary Sjogren's
syndrome occurs with rheumatic diseases such as rheumatoid
arthritis, systemic lupus erythematosus and scleroderma.
Primary Sjögren's
syndrome occurs by itself and is not associated with other
diseases.
Sjögren's
syndrome is an autoimmune disease in which lymphocytes invade
and damage moisture-producing glands, primarily of the eyes
and mouth, preventing them from producing tears and saliva,
respectively (Arthritis Foundation, 1997).
In addition to
the physical and functional problems associated with microstomia,
there are social and relational side-effects. For example,
eating and speaking generally imply human interaction; when
these functions are compromised, so too is one's interactional
ease and capability.
The effects of
microstomia may also limit or interfere with acts of affection
and sexuality—vital aspects of human relationships.
In essence, this
particular symptom has the capacity to compromise an individual
on many different levels – physically and emotionally;
personally and interpersonally.
Scleroderma
En Coup De Sabre
As noted above, linear scleroderma en coup de sabre
is the localized variety of scleroderma that produces facial
changes. "En coup de sabre" is a French term which
means "cut of the sword." It is so named because
its deep lesion or scarring resembles a saber wound.
Scleroderma en
coup de sabre is confined to one side of the face and
cranium (head) and may occur with or without facial hemiatrophy.
The disorder known
as Progressive Hemifacial Atrophy (PHA), or Parry-Romberg
syndrome, has considerable clinical overlap with scleroderma
en coup de sabre. In fact, there is great debate in
the literature as to whether these two syndromes are separate
entities or manifestations of the same or related pathogenic
processes (Lehman, 1992).
There are three
phases that generally accompany scleroderma en coup de
sabre: the initial inflammatory phase; induration; and
subsequent atrophy of the skin and underlying tissues, including
fat and fascia (fiberlike connective tissue).
In severe forms
of this disorder, especially those originating in childhood,
muscle atrophy, cartilage changes, and impaired bone development
in the area of the lesion may occur as well (Lakhani &
David, 1984; Mayes, 1999).
In the area of
the lesion, there also may be accompanying hair loss, including
hair on the scalp, eyebrow, eyelashes, and facial hair. There
may also be hyperpigmented borders and telangiectasias in
the area of the lesion (David, Wilson, & Woo, 1991).
The most common
early sign of scleroderma en coup de sabre is a painless
furrow or indentation near the midline of the face, usually
on the forehead or at the frontal hairline, which marks the
boundary between normal and atrophic tissue (Mayes, 1998;
Munchnick, Aston, & Rees, 1979).
The line of thickened
skin on the affected side of the face may spread to involve
the cheek area, jaw, chin, neck, and upper extremity.
It is interesting
to note that in a number of cases of scleroderma en coup
de sabre there have been reports of central nervous system
involvement (e.g. seizures, epilepsy, focal deficits, headaches),
as well as various ocular or eye changes on the affected side
of the face (Lederman, 1984).
Ocular changes
may include diminished vision, central corneal thickness,
dilated pupil, Horner's syndrome, mydriasis, heterochromia
iridis, and uveitis (Johnson & Kennedy, 1969).
Author's
Personal Experience
Scleroderma and its accompanying facial changes became a particular
topic of interest to this author because of my own experience
with scleroderma en coup de sabre with hemifacial atrophy.
This interest, along with a curious lack of available information
on the psychosocial impact of scleroderma-related facial changes,
both in the scientific and lay literature, eventually led
to my decision to write my doctoral dissertation on this topic.
At the time I began
to research my dissertation in 1994, I had lived with scleroderma
and its accompanying facial changes for 27 years. I was approximately
12 or 13 when the illness began to manifest on the left side
of my face, but I was not diagnosed until I was 18, the day
before I was to leave for my first year of college.
Not unlike the
case of many other individuals diagnosed with scleroderma,
and for the majority of participants in my study, this illness
and its accompanying facial changes were so insidious at first,
barely discernible to the human eye, and then enigmatic, puzzling
doctor after doctor, that it took years to manifest and diagnose.
In my case, the
doctor who rendered the diagnosis was a dermatologist whom
my family physician had brought in for a consultation. After
months of visiting doctors and undergoing various exams and
procedures, it took the dermatologist less than a minute to
make the correct diagnosis.
My illness was
typical in its initial manifestation, although I was not aware
of it when it began. However, some years after I was diagnosed,
I surveyed photographs taken in middle school and it was then
that I noticed the classic early sign of en coup de sabre:
the indentation on the forehead.
I also believe
my illness began around that time because research reveals
a higher incidence of this illness for females at the time
of menarche (first menstruation), pregnancy, and menopause
(Chazen, Cook & Cohen, 1962); and I had started my menses
when I was approximately 12 1/2 years old.
I was 17 when a
single plaque, later diagnosed as morphea, appeared on the
jaw of the affected side. Over the next few months I experienced
severe facial pain, headaches, and had difficulty opening
my mouth.
Although the doctors
were still unable to diagnose my illness, they prescribed
pain medication and valium to treat the symptoms. Several
months later the hair on my eyebrows began to fall out, as
did portions of my eyelashes.
A small bald spot
appeared on my head (which today has progressed to form two
deep furrows), and over the next few years progressive hemifacial
atrophy developed. I was in my mid-thirties when I also noticed
several eye changes; these changes may have occurred years
earlier and gone unnoticed.
Although I have
experienced functional problems and physical pain over the
years as a result of scleroderma-related facial changes, the
most difficult challenge for me, by far, has been dealing
with the personal and interpersonal ramifications of these
changes.
The age at which
these changes began to appear perhaps made this experience
particularly difficult, given the fact that adolescence and
young adulthood is a time when one is searching for her identity
and wanting to "fit in" and be accepted by her peers.
At the very least,
I was confused about my life, and this newly added dimension
made me feel and look different. Having never met another
person with scleroderma and its accompanying facial changes
during those years increased my sense of confusion and isolation.
It was in the process
of interviewing the participants for my study, however, and
listening to their stories, that my own experience found its
voice and validation.
Study
Participants
Although my personal history with scleroderma and the lack
of available research on scleroderma-related facial changes
laid the foundation for my work, several other factors helped
me to further refine my topic and choose the individuals I
eventually solicited as participants.
I interviewed 13
women for my study, one of whom had scleroderma en coup
de sabre with hemifacial atrophy and served as a participant
in the pilot study; 12 of whom had contracted the systemic
form of this illness and had lived with scleroderma-related
facial changes for at least two years.
I chose only women
participants for three reasons:
- Until recently, the vast majority of medical research
has overwhelmingly focused on illnesses that affect males;
and medical studies, in general, have recruited primarily
white males as participants;
- Incidence studies demonstrate that females develop systemic
and localized scleroderma at least three to eight times
more often than males, although Mayes (1999) reports that
linear scleroderma tends to affect females and males equally.
- I hypothesized that, to a greater extent than men, women
who contract an illness that causes an alteration in facial
appearance would more likely experience, as I had, a profound
reaction on both personal and interpersonal levels, especially
given that women are relationally oriented and live in a
society that places tremendous value on our physical beauty,
especially facial beauty.
At the time of
the interviews, which took place in the winter and spring
of 1999, the participants in my study were between the ages
of 34 and 62. Seven of the participants were in their 50's;
two were in their 30's; two were in their 40's; and one participant
was in her 60's. Of the 12 female participants, nine were
Caucasian; two were African-American; and one was Latina.
With respect to relational status, all of the women were heterosexual
and had been married at least once; eight were currently married;
three were single; and one was widowed.
With respect to
the physical effects of the illness, 10 of the 12 participants
had facial changes that resembled, to one degree or another,
what several participants referred to as the "scleroderma-look"—what
was described earlier in this article by Clements and Medsger
(1996) as the "characteristic 'pinched nose' or 'mauskopf'
appearance" (p. 390). During the interview process, Kara
and Cheryl, among others, made reference to this particular
aspect of their appearance:
Cheryl:
Seeing [a well-known spokesperson with scleroderma], seeing
her face, a picture of her and her facial involvement, I
thought, "Oh, I have that same look."
Kara:
When I get my [scleroderma] newsletter, I look at the pictures
[of people with scleroderma] ... because to me it's almost
like Down's Syndrome; there's certain characteristics. I
think I have the same characteristics ...
With respect to
symptomatology, the first and most predominant symptoms reported
by 10 of the 12 participants in terms of their facial changes
were skin tightening and fibrosis. As noted in the testimonies
below, four of the participants and one participant's doctor
described the effects of these symptoms as resembling a "face
lift."
Millie:
I could feel tightening, more tightening ... I would tell
my friends, "I am getting a face-lift!"
Kara:
At first [the facial changes] were like a gentle face lift.
Debbie:
It's like I had a face lift ... [my face] sunk in. See,
it's just like somebody went in and stuck a needle and sucked
the fat out. Almost like cosmetic surgery.
Laura:
[My doctor] said, "You're getting a free face lift."
All of the participants
reported experiencing various functional difficulties or disabilities
due to their facial changes. The vast majority of functional
problems were due to the effects of microstomia. All but one
of the participants reported developing microstomia, and all
of the participants with microstomia reported difficulty with
eating.
Go
to Part 2 of "Losing Face."
Go to Part 3 of "Losing Face."
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