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Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 446
Bilateral Morphea en coup de sabre. A Rare Presentation

1 Department of Dermatology, Faculty of Medicine, Universidad de Chile, Santiago, Chile
2 Dermopathology Section, Pathology Service, Hospital Clínico Universidad de Chile, Santiago, Chile

Date of Web Publication17-Sep-2021

Correspondence Address:
Pablo Vargas-Mora
Department of Dermatology, Faculty of Medicine, Universidad de Chile, Santiago
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_381_20

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How to cite this article:
Vargas-Mora P, Orlandi D, Aranibar L, Morales C. Bilateral Morphea en coup de sabre. A Rare Presentation. Indian J Dermatol 2021;66:446

How to cite this URL:
Vargas-Mora P, Orlandi D, Aranibar L, Morales C. Bilateral Morphea en coup de sabre. A Rare Presentation. Indian J Dermatol [serial online] 2021 [cited 2023 Feb 8];66:446. Available from:

Morphea en coup de sabre (MCS) is an infrequent sub-type of localized linear scleroderma. It is clinically characterized by a frontal linear sclerotic plaque, usually unilateral. It usually starts during the first decade of life, predominantly in females, and follows a slow but progressive course.[1],[2]

We report a case of bilateral MCS, given its exceptional presentation.

A 5-year-old girl presented to us with bilateral erythematous lesions on the frontal region of 6-month duration. On physical examination, a left frontoparietal erythematous-orangey linear plaque was evident. It was slightly atrophic and compromised the supraciliary and upper palpebral areas. It was weakly delimited and associated with alopecia of the scalp. On the right frontal region there was a lesion of similar characteristics of a smaller size, extending from the ipsilateral upper eyelid to the frontal zone, with partial alopecia on the end of the eyebrow and upper eyelashes, but without compromise of the scalp [Figure 1].
Figure 1: (a) Erythematous and atrophic linear frontal plaque. (b) Left frontoparietal linear plaque with localized alopecia of the scalp. (c) Right frontal lineal plaque with partial alopecia of the eyebrow and upper eyelashes

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A color Doppler ultrasound was carried out as we suspected a diagnosis of either MCS or focal facial dermal dysplasia (FFDD). We saw signs of dermal and subcutaneous inflammation suggestive of morphea, with greater activity in the external eyebrow and upper eyelid regions [Figure 2]. Exome sequencing was also carried out and found to be negative. A histopathology study revealed superficial and deep perivascular lymphoplasmacytic fibrosing dermatosis, compatible with morphea [Figure 3]. Treatment began with methotraxate 0.3 mg/kg/week, with a significant clinical and ultrasound improvement and inactivity of the lesions after 6 months of treatment.
Figure 2: (a and b) Color Doppler ultrasound: decreased dermal echogenicity in ciliary regions, lateral segments and upper eyelids, with increased subcutaneous echogenicity. Greater activity signs in the external eyebrow and upper eyelid regions

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Figure 3: (a) 10x H/E. Superficial and deep perivascular infiltrate with dermal fibrosis. (b) 40x, H/E. Inflammatory cell infiltrate composed of lymphocytes and plasma cells

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Bilateral MCS is an extremely rare entity. In a retrospective study of 54 patients, only 4 cases were found with bilateral compromise,[2] which together with another 6 isolated cases reported,[3],[4] add up to a total of 10 cases published in the literature; three of them suffering also from Parry-Romberg syndrome on one side of the face and MCS on the other. It has been observed that some children that presented with typical MCS evolved into the Parry-Romberg syndrome after several years.[4] A prolonged follow-up period is therefore vital as it is often difficult to distinguish involuted MCS from this syndrome: this suggests that they are part of the spectrum of the same disease.[2],[4]

In the present case, the extension of the lesions to below the eyebrows, with a greater activity on the upper eyelids shown by ultrasound, is notable; this is something unusual in MCS. Because of this clinical presentation the differential diagnosis from FFDD was important. The latter include bitemporal or preauricular atrophic plaques, especially in the type 1 (Brauer syndrome) which has no facial dysmorphic findings; but also type 2 (Brauer-Setleis syndrome) and type 3 (Setleis syndrome), which includes in its findings cranial-facial dysmorphism associated with the loss of eyebrows and/or eyelashes.[5] Because of this, faced with a suspicion of bilateral MCS, as well as cranial nuclear magnetic resonance and evaluation by neurology and ophthalmology, it is important to carry out a genetic study to rule out these other syndromes that may be very similar clinically.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Kunzler E, Florez-Pollack S, Teske N, O'Brien J, Prasad S, Jacobe H. Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort. J Am Acad Dermatol 2019;80:1664-70.e1.  Back to cited text no. 1
Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-63.  Back to cited text no. 2
Rai R, Handa S, Gupta S, Kumar B. Bilateral en coup de sabre-a rare entity. Pediatr Dermatol 2000;17:222-4.  Back to cited text no. 3
Gambichler T, Kreuter A, Hoffmann K, Bechara FG, Altmeyer P, Jansen T. Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications. BMC Dermatol 2001;1:9.  Back to cited text no. 4
Lee BH, Aggarwal A, Slavotinek A, Edelmann L, Chen B, Desnick RJ. The focal facial dermal dysplasias: Phenotypic spectrum and molecular genetic heterogeneity. J Med Genet 2017;54:585-90.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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