A single frontal plaque revealing coup de sabre scleroderma in an adult male

Kacimi Alaoui Imane, Zakia Douhi, Sara El-Ammari, Meryem Soughi, Sara Elloudi, Hanane Baybay, Fatima-Zahra Mernissi

Department of Dermatology, University Hospital Hassan II, Fes, Morocco

Corresponding author: Kacimi Alaoui Imane, MD, E-mail: kacimiimane92@gmail.com

How to cite this article: Imane KA, Douhi Z, El-Ammari S, Soughi M, Elloudi S, Baybay H, Mernissi F-Z. A single frontal plaque revealing coup de sabre scleroderma in an adult male. Our Dermatol Online. 2023;14(e):e52.
Submission: 01.02.2023; Acceptance: 07.09.2023
DOI: 10.7241/ourd.2023e.52

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© Our Dermatology Online 2023. No commercial re-use. See rights and permissions. Published by Our Dermatology Online.

Scleroderma characterised by linear “coup de saber” is a variant of localized scleroderma that occurs in the hemiface, although it can be bilateral [1]. First described by Addison in 1854, it peaks in the fifth decade of life. Children are often diagnosed between the ages of 2 and 14 years, and females are more likely to be affected than males [2]. In addition to scarring on the forehead and scalp, the disease causes atrophy of the skin and subcutaneous tissues, including muscles, tendons, and bones [3]. Treatment relies primarily on immunosuppressants and corticosteroids [1,2].

This is a 38-year-old female patient, chronic smoker, who consulted for a hypopigmented lesion of the forehead, evolving for one year, progressively increasing in size, painless and not pruritic. Dermatologic examination revealed a well-limited, roughly rounded, hypochromic plaque with a smooth, slightly atrophic surface, 3 cm in size, located on the forehead (Fig. 1a). Dermatoscopy showed a slightly erythematous background with perifollicular pigmentation (Fig. 1b). Upon examination, a skin biopsy revealed plaque morphea. The patient was put on Calcipotriol + Betamethasone for 4 months. The patient was put on Calcipotriol + Betamethasone for 4 months. The plaque worsened and increased in size, becoming more depressed, with a sclerosed surface responsible for a non-scarring alopecic plaque with a positive traction sign at the periphery (Fig. 1c). In addition, the patient complained of headaches and visual fog for 4 months, so a craniofacial MRI showed no abnormalities. The patient was then put on a bolus of solumedrol and injectable methotrexate (15 mg/week) with positive progress.

Figure 1: (a) Well-limited hypochromic plaque, roughly rounded located on the right forehead. (b) Dermatoscopy showed a slightly erythematous background, with perifollicular pigmentation. (c) A depressed linear plaque, well-limited and sclerosed, located on the right side of the forehead.

Although coup de sabre morphea is a rare entity, it requires more research to identify the exact pathophysiology, as well as effective therapeutic options, for improving aesthetic outcomes and preventing long-term psychological damage.


The examination of the patient was conducted according to the principles of the Declaration of Helsinki.


1. Careta MF, Romiti R. Localized scleroderma:clinical spectrum and therapeutic update. An Bras Dermatol 2015;90:62-73.

2. Ayoub R, Saba SC. Treatment of linear scleroderma “En coup de Sabre“with single-stage autologous fat grafting:A case report and review of the literature. J Cosmet Dermatol. 2021;20:285-9.

3. Amaral TN, Marques Neto JF, Lapa AT, Peres FA, Guirau CR, Appenzeller S. Neurologic involvement in scleroderma en coup de sabre. Autoimmune Dis 2012;2012:1- 6.


Source of Support: This article has no funding source.

Conflict of Interest: The authors have no conflict of interest to declare.

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