The Romberg's Connection 2009 Survey Results

En coup de sabre

Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
1. Please select the location of En coup de sabre:
Location Responses
(159)		 % of Number responding
Cheek20
22%
Forehead56
62%
Chin33
37%
From scalp to
chin, right side		9
10%
From scalp to
chin, left side		13
14%
Tongue14
16%
Other
(Specify)		 14
16%
Number responding = 90 / percent responding = 63% of total respondents (143).
Note: This was a multiple choice question.
This chart has a link to additional information.




Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
2. Where did it start:
% of
Total
Responses		 Responses
(83)		 Where did it start:
1.2%1 Around 1997
1.2%1 Around lip and nose areas
1.2%1 Around the eyes and drooping eyelid and whole of left side of face
1.2%1 Blue discoloration
1.2%1 Both face and back progression started in the same week after accidents.
1.2%1 Center of chin
7.2%6 Cheek
4.8%4 Chin
1.2%1 Chin / right side of face in general
1.2%1 Chin and cheek
1.2%1 Chin with a dark spot glowing spot
1.2%1 Crown of head
1.2%1 Discoloration under left eye and on stomach
1.2%1 Discoloring of neck, hardness of skin on cheek
1.2%1 Don't know for sure. Looking at it, I would guess in the forehead.
1.2%1 Droopy eye lid, white patch of hair
1.2%1 Ear
1.2%1 En coup de sabre was first noticed on left side of face.
1.2%1 Eye and chin
1.2%1 Eye brows
1.2%1 Face/ cheek
12.%10 Forehead
7.2%6 Forehead-mid
1.2%1 From between my eyes to the top of my forehead.
1.2%1 Hair loss on the eyebrow and a white spot on the forehead above the eyebrow.
1.2%1 I believe it started in the cheek and has now progressed to the temple.
1.2%1 In her hair
1.2%1 In my upper lip and eye
1.2%1 Initially started at left upper lip
1.2%1 Just below bottom lip
1.2%1 Just noticed a ridge running down the left side of my face one day, and there wasn't one on the other side.
1.2%1 Left arm
1.2%1 Left chin area- near mouth
1.2%1 Left upper lip
1.2%1 lower cheek
1.2%1 Mid forehead and proceeded into scalp with bone loss in jaw
1.2%1 My cheek, followed by the eye
2.4%2 Neck
3.6%3 Not sure
1.2%1 On my back
1.2%1 On the chin and on the scalp
1.2%1 On upper lip
1.2%1 Possible chin
1.2%1 Probably in the cheek or forehead - possibly with a discolored spot on my back which then atrophied
1.2%1 Probably loss of hair
1.2%1 Right side of chin
1.2%1 Right side of lower back
2.4%2 Scalp
1.2%1 Scalp with a white spot and hair loss on left temple
1.2%1 Small quarter size blemish on left jaw bone area
1.2%1 Temple / cheek
1.2%1 The lower part of the left side of my lip in the corner
1.2%1 Top of head (scalp)
1.2%1 Under eye
1.2%1 Under right eye, looked like a red rash
1.2%1 Upper right side of forehead
1.2%1 With a line of scleroderma on the cheek and ear
Number responding = 83 / percent responding = 58% of total respondents (143).




Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
3. At what age:
Age Responses
(84)		 % of Number responding
45 - 491
1%
40 - 440
0%
35 - 394
5%
30 - 341
1%
25 - 295
6%
20 - 245
6%
15 - 195
6%
10 - 1419
23%
5 - 922
26%
1 - 417
20%
At
birth		1
1%
Don't
Know		4
5%
Number responding = 84 / percent responding = 59% of total respondents (143).




Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
4. Where did it progress to:
% of
Total
Responses		 Responses
(79)		 Where did it progress to:
1.3%1 Around the chin up into the cheek and now into the forehead.
1.3%1 Bottom of chin
1.3%1 Cheek
1.3%1 Cheek, ear, nose eyelid
1.3%1 Cheek, forehead, chin, face, body
1.3%1 Cheek, right nostril, recently forehead
1.3%1 Cheek/scalp
5.1%4 Chin
1.3%1 Chin, eye
1.3%1 Chin and tongue
1.3%1 Chin, jaw, cheek
1.3%1 Complete left chin up to near by the ear
1.3%1 Down and across right side of face and back in my scalp
1.3%1 Down between her eyes
1.3%1 Down forehead and around left ear and then left cheek
1.3%1 Down into neck area, up around nose, around eye, cheek area, lips-right forehead- eyebrow
1.3%1 Down to the bottom of my forehead
1.3%1 Entire right side of face and under chin.
1.3%1 Entire right side of face up into hair
1.3%1 Entire side of face and spots of atrophy on body
1.3%1 Everything!
1.3%1 Eye
1.3%1 Eye lid
1.3%1 Eye, scalp, chin
1.3%1 Face
1.3%1 Face, neck, arm, chest wall, leg
3.8%3 Forehead
1.3%1 Forehead, chin
1.3%1 Forehead, eye, chin, lips, ear
1.3%1 Forehead, lips, nose, tongue, eye
1.3%1 Forehead, then cheek
1.3%1 From cheek to chin
1.3%1 From the left eye, to the left side of the crown of my head, creating a big dent.
1.3%1 Front scalp area to chin.
1.3%1 Full side of the cheek, temple
1.3%1 Further onto lip and into cheek and then upper forhead
1.3%1 Head and chin
1.3%1 Indentation on right side of scalp and loss of hair
1.3%1 It progressed all over the left side of my skull.
1.3%1 Left eye, left cheek then chin
1.3%1 Left leg got a slight indent following pain, then chin showed change-pigment changes on face.
1.3%1 Left side of face, eye, nose, forehead, scalp, left breast, back, stomach
1.3%1 Length of face
1.3%1 Lip, nose
1.3%1 MOVE DOWN TOWARD THE BRIDGE OF MY NOSE AND UPWARD TOWARD MY SCALP. BY AGE 7, IT HAD INDENTED AND MOVED AN INCH OR SO INTO MY SCALP CAUSING ALOPECIA. MOVING DOWNWARD THE HAIR ON ONE SIDE OF MY EYEBROW IS GONE AND ALSO SOME OF MY EYELASHES. MY NOSE IS VERY ASYMETRICAL.
1.3%1 More atrophy of the cheek and chin, then temple and forehead
1.3%1 Mostly up and back into the skull
1.3%1 My whole left side of face, arm, & leg
1.3%1 Nose
1.3%1 Nose, inside mouth
1.3%1 Nose, chin
1.3%1 PRS symptoms
1.3%1 Right cheek and then right upper thigh
2.5%2 Right side of face
1.3%1 Same
1.3%1 Same spot
2.5%2 Scalp
1.3%1 Scalp to chin to forhead to tongue
1.3%1 Stayed in area
1.3%1 Stayed in scalp
1.3%1 Still ongoing
1.3%1 Teeth
1.3%1 The side of face, in different areas on face
1.3%1 The temple
1.3%1 To cheek and forehead
1.3%1 To scalp and through eye, along nose to chin
1.3%1 To the back of my head and also to my nose
1.3%1 Top of chin
1.3%1 Top of skull
1.3%1 Unknown
1.3%1 Up to top of head
1.3%1 Whole left side of face, cheek, side of nose temple
Number responding = 79 / percent responding = 55% of total respondents (143).




Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
5. At what age did it appear to stop:
   Age    Responses
(83)		 % of Number responding
45 - 493
4%
40 - 442
2%
35 - 391
1%
30 - 346
7%
25 - 298
10%
20 - 248
10%
15 - 198
10%
10 - 149
11%
5 - 93
4%
Not
Sure		11
13%
Still
Active		24
29%
Number responding = 83 / percent responding = 58% of total respondents (143).




Page 26. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 1 of 2)
6. Was there any hair loss before or after:
Hair loss Responses
(87)		 % of Number responding
Before10
11%
After20
23%
Before
and After		18
21%
None39
45%
Number responding = 87 / percent responding = 61% of total respondents (143).




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
1. Was there any skin discoloration before or after:
Discoloration Responses
(87)		 % of Number responding
Before25
29%
After15
17%
Before
and After		24
28%
None23
26%
Number responding = 87 / percent responding = 61% of total respondents (143).




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
2. Any medication or procedure that stopped the progression:
% of
Total
Responses		 Responses
(50)		 Any medication or procedure that stopped the progression:
2%1 I WAS GIVEN ANABOLIC STEROIDS.
2%1 I was not diagnosed until I was 23. The progression had stopped and no medication was given.
2%1 I haven't got to be under a doctor for this just got to see a cosmatologist and he couldn't help me.
2%1 We have tried Methotrexate but hard to judge the progession or abatement of PRS.
2%1 Methotrexate and Prednisone oral tablets
Dovonex cream
2%1 On meds now to slow down progression. Do I think they work, no. Methotrexate, folic acid, Prednisolone, Naproxen, Prilosec, Zantac, Keppra, vitamin B6, Albutedol, I think that's all.
2%1 Methylprednisolone and Methotrexate
2%1 I haven't tried any medication specifically for PRS- taking too much as it is for Lupus already! Although, I'm currently on Plaquenil- isn't that a medication that is prescribed for PRS??

I have had a free- flap to face surgery in 1995 using a flap from my scapular area done by Dr. Julian Pribazz of Brigham and Women's Hospital in Boston, MA. I've also had several fat injection surgeries by Dr. Stotland, and he excised my "bald spot" on the en coup de sabre and tried to sew up the "bald spot" in my right eyebrow, but now that's gone. He also rebuilt my left eye socket as it had atrophied, and I had a procedure during which he and another Dr. had moved my left eye up and took a piece of cartilage from my right ear and used it in my lower left eye lid to bring it out, etc. I'm really just a piece of work!!

2%1 Removal of initial affected area on lip and gum, they left area on nose etc.
2%1 Methotrexate & Predisolone, seems like it stops off & on.
2%1 Not sure anything helped.
2%1 High doses of Penicillin for two weeks i. v.
Suppose that it stopped for about 5 years, then started again.
Process of progression is very slowly.
2%1 Steroids were tried when I was first diagnosed, but appeared to have no effect. I have had 3 fat injections, which do not stop the progression but certainly help the aesthetics.
2%1 Steroids
2%1 A cream was applied to the area where it started to burn it down. It was raised and after using the cream, it is now smooth.
2%1 Yes Methotrexate
2%1 Microvascular free flap transfer
2%1 She takes Methotrexate and folic acid, and was on Prednisolone. It has slowed down but did not stop that is what doctors say. I honestly have not noticed it stopping sometimes I think it has gotten worse.
2%1 As far as I can recall I was put onto Diprosone (it was a medication given to leppers).
2%1 None, I have been told there is nothing to stop the progression.
2%1 Methotrexate tablets and IV Methylprednisolone
2%1 Was on Doxy on and off for lyme. During that, changes appeared to arrest. Stopped looking, although fasciculations continued body-wide. Was told they were benign fasciculations. Then went thru stressful period (still in it) and noticed chin changes. Had been off Doxy for a year by then.
2%1 I take extra vitamin D, which I think helps. Also an aspirin per day for the vascular dilation to the area.
54%27 None
Number responding = 50 / percent responding = 35% of total respondents (143).




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
3. Please comment on effectiveness of this medication or procedure.
% of Number responding Responses
(27)		 Effectiveness of Medication or Procedure
3.7%1 A picture is worth 1000 words. I'll post one for you to decide. I think that the free flap has held the best so far, hard saying about the rest- seems as if the PRS just keeps on taking back what has been done. That, or i'm just going to either keep on having surgery over and over, or just have to like what I look like no matter what it is.
3.7%1 During this time I had weekly blood counts. Whether it was this medication that halted the atrophy or whether it just did not progress any further one would never know.
3.7%1 Ear
3.7%1 First dose of oral steroids had a dramatic effect on eye inflammation. Ptosis was gone immediately, and redness of conjuctiva disappeared. Any time we have tried to wean her off the small maintenance dose of oral steroids, inflammation returns.
3.7%1 Hard to say for sure.
3.7%1 I don't know what to think.
3.7%1 I WAS GIVEN ANABOLIC STEROIDS. THEY CAUSED THEIR OWN PROBLEMS
3.7%1 It seemed to stop advancement nose continued to regress but the area affected didn't continue to grow.
3.7%1 It seemed to stop or at least slow the progression.
3.7%1 It was very effective in halting the disease and in the reconstruction of my face.
18.5%5 N/A
3.7%1 No medication or procedure was effective in treating Parry-Romberg.
3.7%1 No meds
3.7%1 Not effective in my opinion.
3.7%1 Not helpful
3.7%1 See above
3.7%1 Seems to be inhibiting further progression.
3.7%1 She is currently taking Prednisone and Methotrexate for treatment.
3.7%1 Sometimes I wish I would have never put her through all these medications. She gaines a lot of weight with the Prednisolone and now has asthma, sinus problems, her heart races. She didn't have these problems before.
3.7%1 The lines of Scleroderma are softer and brighter, the regression of half face progresses.
3.7%1 The liquid silicon injections helped fill in partial areas of face, but have left her with problems with infections and spaces between tissue and treated areas.
3.7%1 The steroids were ineffective.
3.7%1 Told me to use a topical cream, but it didn't stop it.
3.7%1 Very effective
Number responding = 27 / percent responding = 19% of total respondents (143).




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
Combine the "Medication or Procedure" (Page 27.2) response with the "Effectiveness" (Page 27.3) response.
Medication or Procedure (Page 27.2) Effectiveness of Medication or Procedure (Page 27.3)
NO I WAS GIVEN ANABOLIC STEROIDS. THEY CAUSED THEIR OWN PROBLEMS
I haven't got to be under a doctor for this just got to see a cosmatologist and he couldn't help me. Told me to use a topical cream, but it didn't stop it.
Methylprednisolon and Methotrexate The lines of Scleroderma are softer and brighter, the regression of half face progresses.
None None
I take extra vitamin D, which I think helps. Also an aspirin per day for the vascular dilation to the area. Hard to say for sure.
None n/a
Microvascular free flap transfer It was very effective in halting the disease and in the reconstruction of my face.
She takes Methotrexate and folic acid, and was on Prednisolone. It has slowed down but did not stop that is what doctors say. I honestly have not noticed it stopping sometimes I think it has gotten worse. Sometimes I wish I would have never put her through all these medications. She gaines a lot of weight with the Prednisolone and now has asthma, sinus problems, her heart races. She didn't have these problems before.
Removal of initial affected area on lip and gum, they left area on nose etc. It seemed to stop advancement, nose continued to regress but the area affected didn't continue to grow.
None No medication or procedure was effective in treating Parry-Romberg.
Methotrexate and Prednisone oral tablets Dovonex cream Seems to be inhibiting further progression.
Yes Methartrexate Very effective
No N/A
Steroids were tried when I was first diagnosed, but appeared to have no effect. I have had 3 fat injections, which do not stop the progression but certainly help the aesthetics. The steroids were ineffective.
Steroids First dose of oral steroids had a dramatic effect on eye inflammation. Ptosis was gone immediately, and redness of conjuctiva disappeared. Any time we have tried to wean her off the small maintenance dose of oral steroids, inflammation returns.
Was on Doxy on and off for Lyme disease. During that, changes appeared to arrest. Stopped looking, although fasciulations continued body-wide. Was told they were benign fasiculations. Then went thru stressful period (still in it) and noticed chin changes. Had been off Doxy for a year by then. I don't know what to think.
We have tried Methotrexate but hard to judge the progession or abatement of PRS. see above
Methotrexate tablets and IV Methylprednisalone It seemed to stop or at least slow the progression.
"I haven't tried any medication specifically for PRS- taking too much as it is for Lupus already! Although, I'm currently on Plaquenil- isn't that a medication that is prescribed for PRS?? I have had a free- flap to face surgery in 1995 using a flap from my scapular area done by Dr. Julian Pribazz of Brigham and Women's Hospital in Boston, MA. I've also had several fat injection surgeries by Dr. Stotland, and he excised my "bald spot" on the En coup de sabre and tried to sew up the "bald spot" in my right eyebrow, but now that's gone. He also rebuilt my left eye socket as it had atrophied, and I had a procedure during which he and another Dr. had moved my left eye up and took a piece of cartilage from my right ear and used it in my lower left eye lid to bring it out, etc. I'm really just a piece of work!!" A picture is worth 1000 words. I'll post one for you to decide. I think that the free flap has held the best so far, hard saying about the rest- seems as if the PRS just keeps on taking back what has been done. That, or i'm just going to either keep on having surgery over and over, or just have to like what I look like no matter what it is.
On meds now to slow down progression. Do I think they work, no. Methotrexate, folic acid, Prednisolone, Naproxen, Prilosec, Zantac, Keppra, vitamin B-6, Albutedol, I think that's all. Not effective in my opinion.
No N/A
Not sure anything helped The liquid silicon injections helped fill in partial areas of face, but have left her with problems with infections and spaces between tissue and treated areas.
"I don't think I have any discoloration other than the red chapped skin.......not sure if that's associated with it or not.......No meds." No meds
As far as I can recall I was put onto Diprosone (it was a medication given to leppers). During this time I had weekly blood counts. Whether it was this medication that halted the atrophy or whether it just did not progress any further one would never know.
No Not helpful
Number responding = 20 / percent responding = 14% of total respondents (143).
Note: Had to have answered both 27.2 and 27.3 to be included here.




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
4. Please rate the severity of the En coup de sabre:
Rating Responses
(86)		 % of Number responding
1 (very mild)5
6%
2 (mild)20
23%
3 (moderate)38
44%
4 (severe)19
22%
5 (very severe)4
5%
Number responding = 86 / percent responding = 60% of total respondents (143).




Page 27. III. Associated Symptoms (page 2 of 4) cont.: En coup de sabre (page 2 of 2)
5. Comments for En coup de sabre:
Comments
(23)
The en coup de sabre start of indention was at the site where i got struck in the face. The hole in right side of my back also started at place I was struck. I still have a small white scar there.
IT IS A VIAL THING THAT HAS MADE ME SELF-CONSCIOUS ALL MY LIFE. I'VE NEVER BEEN ABLE TO PULL MY HAIR BACK BECAUSE OF THIS HUGE INDENTATION AND SCAR. ALSO, WHERE THE FAT AND BONE ARE AFFECTED IT IS VERY TENDER TO THE TOUCH.
To me it looks mild on my forehead.
It is like the dividing line between my Rombergs side and the normal side.
I have become very uncomfortable and self-conscious about my forehead now. I feel like it is drastic and wear my bangs down to cover it every day. I feel like it is "severe" but I have seen pictures of other people who were much worse... so I feel like severe would not be appropriate to write, compared to some others.
I say moderate because I don't know what is mild and what is severe.
It seems mild compared to others. I cover my forehead with hair (bangs). I have no masseter muscle on my left cheek. (MRI shows it missing).
Moderate on top of head, mild to moderate on forehead
Four streaks going down the front of my forehead
Crown of head is severe in flatness... forehead is very mild
Because of the severity of this, I make sure my hair covers my entire forehead and use hairspray to keep it in place.
This disease is very confusing.
From photos I have seen of en coup de sabre, her's seems to be pretty severe and quick spreading.
Stops right below eyebrow level.
From injury site mid-forehead down my forehead, nose and upper lip. About 1.5 cm wide.
My forehead and chin are quite noticeable.
It starts right on the top of my scalp and follows a line down my forehead.
I don't think I should judge the severity because I have nothing to compare to. Seems bad to me.
It was a big ol' dent in my head that used to really bug me because it was bald - I was very self conscious of it unit my Dr. excised it and stitched the skin together so that my hair covered it - now it isn't noticable.. When you push on the dent it causes pain though. Other than that, no problems.
Has a crooked chin at age 8, the doctors indicated PRS but nothing was done as only the chin was affected at the time.
She has very visible deterioration and has physical problems.
On the forehead and the chin.
Chin
Number responding = 23 / percent responding = 16% of total respondents (143).


Disclaimer: Please be advised that everyone's experiences may be different and appropriate treatments may vary. Any medical information that you find on The Romberg's Connection website must NOT serve as a substitute for a consultation with one's personal physician. Our visitors should discuss any specific questions or concerns they may have about Rombergs with health care professionals who are familiar with the specifics of their special case.
As a support group, The Romberg's Connection is unable to offer medical advice to anyone.

Throughout this survey, when we use the term "Rombergs" or "Romberg" we are referring to: "Parry Romberg", "Parry Rombergs", "Parry Romberg Syndrome" and "PRS".

This is an unscientific survey designed to gather information from those who must deal with Rombergs on a daily basis.

Last Updated August 22, 2009