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Morphoea en coup de sabre

Author: Elaine Luther, Medical Student, Ross University School of Medicine, Barbados, West Indies. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. April 2020.


What is morphoea en coup de sabre?

Morphoea en coup de sabre is a variant of linear morphoea (a localised form of scleroderma) that is restricted to the frontoparietal region (forehead). The name ‘en coup de sabre’ (the blow of a sword) derives from the characteristic scar that indents the skin of the scalp and the underlying bone [1]. Morphoea en coup de sabre also can cut into the brain, causing neurological abnormalities and vision problems.

Morphoea en coup de sabre is also known as frontoparietal linear morphoea (American spelling morphea), linear morphoea en coup de sabre, or just 'en coup de sabre'.

Morphoea en coup de sabre

Who gets morphoea en coup de sabre?

Morphoea en coup de sabre mostly occurs in children (67%) [2], with a slight predominance in girls (about 2:1) [3]. The average age of onset is 13.6 years of age [3].

What causes morphoea en coup de sabre?

The exact cause of morphoea en coup de sabre is unknown. It is believed to be an autoimmune inflammatory disease. There is probably a genetic predisposition, although the specific genetic factors are unknown. The trigger initiating the inflammation is usually unknown, but has been reported to include trauma, Lyme borreliosis, radiotherapy, and herpes zoster ophthalmicus. The resulting inflammatory response causes the characteristic damage.

What are the clinical features of morphoea en coup de sabre?

The clinical features of morphoea en coup de sabre evolve over months to years. En coup de sabre begins with a slight hyperpigmented or hypopigmented streak on the forehead. The streak becomes indurated, evolving into a scar. As this happens, the underlying scalp and sometimes even the skull indent. As the scar grows, it reaches past the hairline into the scalp, causing linear cicatricial alopecia.

Morphoea en coup de sabre

What are the complications of morphoea en coup de sabre?

The complications of morphoea en coup de sabre include:

  • Hair loss
  • Scalp atrophy or atrophy of the underlying bone (the calvarium) [4]
  • Progressive hemifacial atrophy (Parry-Romberg syndrome) [3–5]
  • Ipsilateral eye problems (structural and visual abnormalities) [4,6]
  • Neurological abnormalities (eg, seizures and headaches) [4]
  • Facial deformity
  • Reduced self-esteem See psychosocial factors in dermatology.

How is morphoea en coup de sabre diagnosed?

The diagnosis of morphoea en coup de sabre can usually be performed clinically by noting the characteristic clinical features. Dermoscopy shows thickened telangiectatic vessels due to inflammation, fibrosis, and loss of follicles [7].

If necessary, a skin biopsy can distinguish en coup de sabre from similar conditions. The histology shows the classical pathological features of scleroderma [8–11].

Negative tests for anti-endothelial antibodies, antinuclear antibodies, and anti-Scl-70 can rule out other connective tissue diseases [12].

If borreliosis is suspected, a polymerase chain reaction (PCR) for Borrelia DNA is more reliable than testing for serum antibodies or the ELISA/Western blot [12,13].

Imaging may include ultrasound, plain X-rays, and computed tomography (CT) scans. Calvarial and intracranial abnormalities may be investigated with magnetic resonance imaging (MRI), which may show skull abnormalities, focal brain atrophy, calcification, and white matter lesions.

What is the differential diagnosis for morphoea en coup de sabre?

Diagnosis can be difficult during the early phase when the morphoea is no more than a pigmented line. Other conditions confused with morphoea en coup de sabre may include:

What is the treatment for morphoea en coup de sabre?

The goal of treatment is to prevent progression of the en coup de sabre. The most commonly used treatment is a combination of methotrexate with oral corticosteroids [14–16]. There are no published guidelines for treatment of morphoea en coup de sabre, and no standard of care. A wide range of other treatments have been successful in individual case reports.

Corticosteroids

Formulations of corticosteroids used for en coup de sabre include:

Immune modulating agents

Other medications with effects on the immune reaction include:

Phototherapy

Types of phototherapy used for morphoea en coup de sabre include:

Vitamins

Vitamin-based therapy reported of benefit in en coup de sabre includes:

Antibiotics

Antibiotics have antibacterial (eg, targeting borreliosis) and anti-inflammatory (nonspecific) properties.

Reconstructive surgery

Once en coup de sabre is burnt out, the atrophic skin and underlying structures may undergo surgical repair. Techniques may include:

  • Dermal fat graft
  • Implants of bone or synthetic materials
  • Tissue expansion
  • Surgical resection
  • Skin and muscle flap.

What is the outcome for morphoea en coup de sabre?

The natural history of morphoea en coup de sabre is to progress slowly over many years, then to be self-limiting. There are three phases [3,16].

Active phase

The active phase of morphoea en coup de sabre features areas of active sclerosis with erythematous or violaceous borders, with or without expansion of the plaque. The goal of treatment during the active phase is to slow or stop progression with immunosuppressants.

Regression phase

The regression phase of morphoea en coup de sabre retains persistent sclerosis without erythema or a violaceous border. The loss of redness indicates that en coup de sabre is regressing spontaneously or due to treatment.

Burnt-out phase

The burnt-out phase of morphoea en coup de sabre demonstrates no sclerosis and no erythema. The burnt-out phase signals that the course of en coup de sabre is complete, and no further damage will occur. Plastic surgery is usually deferred until the burnt-out phase [17].

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References

  1. Lauesen SR, Daugaard-Jensen J, Lauridsen EF, Kjær I. Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of six patients. Eur Arch Paediatr Dent 2019; 20: 339–50. doi: 10.1007/s40368-019-00427-7. PubMed
  2. Holland KE, Steffes B, Nocton JJ, schwabe MJ, Jacobsen RD, Drolet BA. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics 2006; 117: e132–6. DOI: 10.1542/peds.2005-0470. PubMed
  3. Tollefson MM1, 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. DOI: 10.1016/j.jaad.2006.10.959. PubMed
  4. Chiu YE, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol 2012; 29: 738–48. doi:10.1111/pde.12001. PubMed
  5. Blaszczyk M, Królicki L, Krasu M, Glinska O, and Jablonska S. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol 2003; 30: 1997–2004. PubMed
  6. Fledelius HC, Danielsen PL, Ullman S. Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. Eye (Lond) 2018; 32: 1688–96. doi: 10.1038/s41433-018-0137-9. PubMed
  7. Saceda-Corralo D, Tosti A. Trichoscopic Features of Linear Morphea on the Scalp. Skin Appendage Disord 2018; 4: 31–3. DOI: 10.1159/000478022. PubMed
  8. Taniguchi T, Asano Y, Tamaki Z, Akamata K, Aozasa N, Noda S, et al. Histological features of localized scleroderma 'en coup de sabre': a study of 16 cases. J Eur Acad Dermatol Venereol 2014; 28: 1805–10. DOI: 10.1111/jdv.12280. PubMed
  9. Pierre-Louis M, Sperling LC, Wilke MS, Hordinsky MK. Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. J Cutan Pathol 2013; 40: 580–4. DOI: 10.1111/cup.12124. PubMed
  10. Biswas A, Goh C, Goldberg LJ. Alopecia with perineural lymphocytes: a clue to linear scleroderma en coup de sabre. J Cutan Pathol 2012; 39: 518–20. DOI: 10.1111/j.1600-0560.2012.01889.x. PubMed
  11. Sehgal VN, Svrivastava PB. En coup de sabre. Int J Dermatol 2002; 41: 504-5. DOI: 10.1046/j.1365-4362.2002.01589.x. Journal
  12. Gubertini N, Bonin S, Trevisan G. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis. Dermatol Reports 2011; 3: e27. DOI: 10.4081/dr.2011.e27. PubMed Central
  13. Katz, KA. Frontal linear scleroderma (en coup de sabre). Dermatol Online J 2003; 9: 10. doi: 10.1136/bcr-2012-007837. PubMed Central
  14. Niklander S, Marín C, Martínez R, Esguep A. Morphea "en coup de sabre": an unusual oral presentation. J Clin Exp Dent. 2017; 9: e315–8. DOI: 10.4317/jced.53151. PubMed
  15. Rattanakaemakorn P1,2, Jorizzo JL2. The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype). J Dermatolog Treat 2018; 29: 197–9. DOI: 10.1080/09546634.2017.1341615. PubMed
  16. Albuquerque JVD, Andriolo BNG, Vasconcellos MRA, Civile VT, Lyddiatt A, Trevisani VFM. Interventions for morphea. Cochrane Database of Syst Rev 2019; 7: CD005027. DOI: 10.1002/14651858.CD005027.pub5. PubMed
  17. Bhoyrul B, Clark S. Parry-Romberg syndrome and morphoea en coup de sabre. Arch Dis Child 2019; 104: 400. PubMed

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