Case Reports

En Coup de Sabre

Cutis. 2019 January;103(1):34-36

References

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

2. Picket AJ, Carpentieri D, Price H, et al. Early morphea mimicking acquired port-wine stain. Pediatr Dermatol. 2014;31:591-594.

3. Holland KE, Steffes B, Nocton JJ, et al. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. 2006;117:132-136.

4. Goh C, Biswas A, Goldberg LJ. Alopecia with perineural lymphocytes: a clue to linear scleroderma en coup de sabre. J Cutan Pathol. 2012;39:518-520.

5. Kreuter A. Localized scleroderma. Dermatol Ther. 2012;25:135-147.

6. Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: a review. Orphanet J Rare Dis. 2015;10:39.

7. Amaral TN, Marques Neto JF, Lapa AT, et al. Neurologic involvement in scleroderma en coup de sabre [published online January 27, 2012]. Autoimmune Dis. 2012;2012:719685.

8. Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56:257-263.

9. Zannin ME, Martini G, Athreya BH, et al. Ocular involvement in children with localized scleroderma: a multi-center study. Br J Ophthalmol. 2007;91:1311-1314.

10. Polcari I, Moon A, Mathes EF, et al. Headaches as a presenting symptom of linear morphea en coup de sabre. Pediatrics. 2014;134:1715-1719.

11. Doolittle DA, Lehman VT, Schwartz KM, et al. CNS imaging findings associated with Parry-Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities. Neuroradiology. 2015;57:21-34.

12. Pierre-Louis M, Sperling LC, Wilke MS, et al. Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. J Cutan Pathol. 2013;40:580-584.

13. Thareja SK, Sadhwani D, Alan Fenske N. En coup de sabre morphea treated with hyaluronic acid filler. Report of a case and review of the literature. Int J Dermatol. 2015;54:823-826.

14. Fett N, Werth VP. Update on morphea: part I. epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64:217-228.

15. Christen-Zaech S, Hakim MD, Afsar FS, et al. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008;59:385-396.

16. Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol. 2009;145:545-550.

17. Kurzinski K, Torok KS. Cytokine profiles in localized scleroderma and relationship to clinical features. Cytokine. 2011;55:157-164.

18. Eisendle K, Grabner T, Zelger B. Morphoea: a manifestation of infection with Borrelia species? Br J Dermatol. 2007;157:1189-1198.

19. Gutiérrez-Gómez C, Godínez-Hana AL, García-Hernández M, et al. Lack of IgG antibody seropositivity to Borrelia burgdorferi in patients with Parry-Romberg syndrome and linear morphea en coup de sabre in Mexico. Int J Dermatol. 2014;53:947-951.

20. Miller K, Lehrhoff S, Fischer M, et al. Linear morphea of the forehead (en coup de sabre). Dermatol Online J. 2012;18:22.

21. Hanson AH, Fivenson DP, Schapiro B. Linear scleroderma in an adolescent woman treated with methotrexate and excimer laser. Dermatol Ther. 2014;27:203-205.

22. Zulian F, Martini G, Vallongo C, et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2011;63:1998-2006.

23. Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol. 2005;141:847-852.

24. Weibel L, Sampaio MC, Visentin MT, et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol. 2006;155:1013-1020.

25. Torok KS, Arkachaisri T. Methotrexate and corticosteroids in the treatment of localized scleroderma: a standardized prospective longitudinal single-center study. J Rheumatol. 2012;39:286-294.

26. Nisticò SP, Saraceno R, Schipani C, et al. Different applications of monochromatic excimer light in skin diseases. Photomed Laser Surg. 2009;27:647-654.

27. Zwischenberger BA, Jacobe HT. A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol. 2011;65:925-941.

28. Karaaltin MV, Akpinar AC, Baghaki S, et al. Treatment of “en coup de sabre” deformity with adipose-derived regenerative cell-enriched fat graft. J Craniofac Surg. 2012;23:103-105.

29. Consorti G, Tieghi R, Clauser LC. Frontal linear scleroderma: long-term result in volumetric restoration of the fronto-orbital area by structural fat grafting. J Craniofac Surg. 2012;23:263-265.

30. Cavusoglu T, Yazici I, Vargel I, et al. Reconstruction of coup de sabre deformity (linear localized scleroderma) by using galeal frontalis muscle flap and demineralized bone matrix combination. J Craniofac Surg. 2011;22:257-258.

31. Robitschek J, Wang D, Hall D. Treatment of linear scleroderma “en coup de sabre” with AlloDerm tissue matrix. Otolaryngol Head Neck Surg. 2008;138:540-541.

Comment

Etiology and Presentation
En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead.^7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone.⁷ Although most cases present with a single lesion, multiple lesions can occur.⁸ The exact etiology of this disease remains to be determined but is characterized by thickening and hardening of the skin secondary to increased collagen production.⁷ The incidence of linear morphea ranges from 0.4 to 2.7 cases per 100,000 individuals and is more prevalent in white patients and women.¹⁴ Linear morphea is commonly found in children. Children are more likely to have linear morphea on the face, which can lead to lifelong disfigurement.² Although the disease peaks in the fourth decade of life for adults, most pediatric cases are diagnosed between 2 and 14 years of age.^14-16

Pathogenesis
Clinical and histopathological data suggest that a complex interaction among the vasculature, extracellular matrix, and immune system plays a role in the pathogenesis of the disease. Similar to scleroderma, the CD4 helper T cell may be involved in the fibrotic changes that occur within these lesions.¹⁷ Early in the disease process, T_H1 and T_H17 inflammatory pathways predominate. The late fibrotic changes seen in scleroderma are more associated with a shift to the T_H2 inflammatory pathway.¹⁷ Infection with Borrelia burgdorferi has been implicated abroad, but a large-scale study confirming Borrelia as a pathologic factor within morphea lesions has not been completed to date.^18-20 Some authors believe early lesions of ECDS mimic erythema chronica migrans, with the late lesions resembling acrodermatitis chronica atrophicans.²⁰

Histopathology
Histopathologic findings of morphea tend to vary depending on the stage of the disease. The 2 stages of morphea can be differentiated by the degree of inflammation present histologically.^14,21 The early phase of morphea primarily affects the connective and subcutaneous tissue surrounding eccrine sweat glands.^14,21 A dense dermal and subcutaneous perivascular lymphocytic infiltrate with a mixture of lymphocytes, plasma cells, and histiocytes is commonly observed.⁵ Later stages of the disease demonstrate densely packed homogenous collagen with minimal inflammation and loss of eccrine glands and blood vessels.^14,21 The adipose tissue is generally replaced by sclerotic collagen, giving the biopsy a squared-off appearance.^5,14

Management
En coup de sabre presents a treatment challenge. In active lesions, topical or intralesional corticosteroids are considered treatment of choice.⁵ Methotrexate has proven useful in the treatment of acute and deep forms of linear morphea. A study examining methotrexate in juvenile localized scleroderma, with the majority of patients having the linear subtype, revealed that methotrexate is both efficacious and well tolerated.²² Other reports in the literature reveal efficacy with the use of intravenous corticosteroids and methotrexate combination therapy for treatment of morphea.^23,24 A longitudinal prospective study examining the use of high-dose methotrexate and oral corticosteroids for the treatment of localized scleroderma yielded positive results, with patients showing clinical improvement within 2 months of initiation of combination therapy.²⁵ Other treatments include excimer laser; calcipotriene and tacrolimus; and surgical approaches such as autologous fat grafting, grafting with muscle flaps, and tissue inserts.^21,26-31 In addition, patients can choose to forego therapy, as was the case with our patient.

Conclusion

En coup de sabre is a rare subtype of linear scleroderma that is limited to the ipsilateral scalp and face predominately in children and women. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. Current treatment recommendations include topical, intralesional, and oral corticosteroids; methotrexate; and surgical grafts. Although ECDS is a rare entity, more intensive research is needed on the exact pathophysiology and effective treatment options that focus on improving the cosmetic outcome in these patients. Cosmesis is the primary concern in patients with ECDS and should be managed early and appropriately to prevent long-term psychological sequelae.