En coup de sabre in a young woman

presented by

Henry Foong, FRCP

Ipoh, Malaysia

September 3, 2008

Abstract 28 yo woman presented with linear scleroderma (en coup de sabre) on the forehead
28 yo woman
2 years
Scalp and forehead

A 28-year-old woman presented with a 2-year history of progressive, hyperpigmented, indented lesion on her forehead that extended into her frontal scalp. This apparently started during her 2nd pregnancy. She had no headaches or seizures. There is no history of Lyme disease. She was otherwise well. No family history of auto immune disease.

Physical Examination

A linear, hyperpigmented, atrophic, depressed plaque was present over the left paramedian forehead, extending from within the frontal scalp to below the supraorbital ridge. A linear patch of scarring alopecia was seen in the scalp plaque. A similar but smaller depressed hyperpigmented plaque was noted on the left forehead. Ophthalmic examination was normal.


Laboratory Data

Blood counts and biochemistry was normal.

Antinuclear antibody: negative

Extractable nuclear antigen serology: negative




Linear Scleroderma (en coup de sabre)

Reasons Presented

En coup de sabre (like the stroke of a sabre) is a rare form of localized linear scleroderma that occurs on the scalp and forehead. Its onset is usually during the first two decades of life. Unlike the gradual spontaneous remissions seen in morphea, linear scleroderma tends to have a longer and occasionally progressive course (1) Some authors suggest scleroderma en coup de sabre occurs along Blaschko lines which suggests that it may arise in a mosaic clone of susceptible cells.(2)

The treatment of en coup de sabre remains difficult. There has been many therapies tried in this condition. Among them are prednisolone, antimalarials, retinoids, penicillamine, penicillins, griseofulvin, calcitriol and methotrexate. There has also been instances where UVA and narrow band UVB phototherapy had shown some good results too. (3, 4)


This is quite a disfiguring condition for the patient.

This patient was started on hydroxychloroquine 400mg daily with topical tacrolimus ointment 0.1%. Would narrow band phototherapy (NB-UVB) help in her case? Is there a role for empirical treatment with oral doxycycline?

If this antimalarial fails, what alternative would be appropriate for her?

if she plans to have a 2nd pregnancy, what treatment would you recommend?

For therapeutic suggestions.


Eubanks LE, et al. Linear scleroderma in children. Int J Dermatol 1996;35:330

Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko's lines. J Am Acad Dermatol 1998;38:366

Brownell I et al. Familial linear scleroderma responsive to antimalarials and narrow band ultraviolet violet B therapy. Dermatol Online J 13(1): 11

Gambichler T, et al. Linear scleroderma 'en coup de sabre' treated with topical calcipotriol and cream psoralen plus ultraviolet A. J Eur Acad Dermatol Venereol. 2003;17:601

Keywords linear scleroderma, en coup de sabre, antimalarials
Comments from Faculty and Members

Patrick Condry M.D., Clinical Asst Professor, Dept Dermatology, University of Rochester, Rochester, NY, USA on September 3, 2008

This is always a tough problem. I had one partial response to cyclosporin which was not in your list of alternatives for therapy. I have never had one completely responded to therapy and many have gone to Plastics for repairs!

Arshad Khan M.D., Consultant Dermatologist, Peshawar, Pakistan on September 3, 2008

I have treated few linear morphoea patients with topical calcipotriol and MTX with good results. If she wants pregnancy, she should be advised to either delay it of just use topicals, which should be safe.

Khaled El-hoshy M.D., Consultant Dermatologist, Troy, Michigan, USA on September 3, 2008

Balloon tissue expansion & closure is one of the options if available. Autologous fat transfer can offer some improvement. The new filler Macrolane, by Q-med may also prove useful.

Omid Zargari, M.D., Consultant Dermatologist, Rasht, Iran on September 4, 2008

Great photos. I had one case with reasonable response to a combination of low dose dexa, topical calcipotriene, pentoxifylline and vitamine E. I don't know which one did the job!, but the outcome was quite acceptable.

Azar Maluki, M.D., Asst. Professor, Dept Dermatology, College of Medicine, University of Kufa, Al-Najaf, Iraq on September 4, 2008

This condition is very challenging for both the patient and doctor. I had treated many such cases with very good impressive results using topical combination of clobetasol prop., retinoid cream 0.1% and alpha hydroxy acid cream . The response was better with the addition of penicillamine caps. and systemic steroids.
On stabilization of the case, many cosmetic alternatives would be available eg. injections of dermal fillers like Hydrogell (Aquamid).
During pregnancy, topical drugs would be a safe option.

Pakhi Pereira, M.D. Consultant Dermatologist, Bangalore, India on September 4, 2008

Is this case still progressing? If she has stabilised, you might try dermal fillers after subscission for a good result.

Nidal Dabbour, M.D. Consultant Dermatologist, Saudi Arabia on September 4, 2008

I think this patient should receive intralesional steroids to be given superficially in the dermis and not in the remaining fat tissue so that the depth of the lesion will not increase. Steroids will halt the inflammatory process which is very mild perivascular B lymphocytes and few plasma cells. Steroids will also dissolve the deposited collagen that replaced the fat tissue. Surgical excesion is a good choice if steroids failed

Bushan Kumar M.D., Professor, Former Head, Dept Dermatology, PGIMER, Chandigarh, India on September 4, 2008

In case of an established sclerotic plaque nothing works except surgical excision. In an evolving lesion, topical calcipotriol and low dose methotrexate given over a period of 6 months to 1 year does help. All other therapies in my view are hardly of any help.

Stelios Minas, M.D., Consultant Dermatologist, Limassol, Cyprus on September 5, 2008

Usually I treat cases like that with inj. Penicillin (1.2 m ui /10 inj)and after that some courses (1-2 months) with plaquenil and trental. As a topical treatment I use microinjection of trental and longidasa . I have seen some effect in some patients. If the effect is insufficient than I refer the patient to plastic surgery for surgical excision.

Jon Robitschek, M.D., Resident, Tripler Army Medical Center, Honolulu, HI, USA on September 5, 2008

Excellent presentation. In our published case we had excellent cosmesis from the use of Alloderm which was followed 18 months postoperatively.

Ref: Robitschek J, Wang D, Hall D. Treatment of linear scleroderma "en coup de sabre" with AlloDerm tissue matrix. Otolaryngol Head Neck Surg. 2008 Apr;138(4):540-1.

Firas Altamimi M.D., Basra Teaching Hospital, Basra, Iraq on September 5, 2008

Nice pictures. The depressed scar need good cosmetic change for her face. I think she could be treated either by topical halofuginone or by CO2 vaporization laser. Topical halofuginone is one of new treatment that is used in local scleroderma and act as inhibitor of type 1 collagen through inhibition of NF-(kappa)B and p38(member of MAPK family). CO2 vaporization is only used if the scleroderma is associated with calcinosis cutis.

Amin Nurul M.D., Professor of Dermatology, Armed Forces Medical College, Dhaka, Bangladesh on September 8, 2008

Excellent photos and presentation. I have treated one case with topical calcipotriol and topical corticosteroid and in another case with topical calcipotriol and pentoxyphylline.

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