Dermatology Online Journal

Linear morphea of the forehead (en coup de sabre)
Kristen Miller MD, Stephanie Lehrhoff MD, Max Fischer MD, Shane Meehan MD, Jo-Ann Latkowski MD
Dermatology Online Journal 18 (12): 22

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

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Abstract

Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. It typically occurs in children although cases of adult-onset ECDS exist as reported here. ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. Sclerosis in ECDS lesions may invade deeply to involve underlying muscle and bone and may exist on the same clinicopathologic spectrum as Parry-Romberg syndrome. Extracutaneous involvement is frequent, with neurologic and ophthalmologic findings occurring most commonly. The etiology of ECDS is unclear but may be autoimmune in origin. The question of whether Borrelia infection also plays a role remains controversial. Current evidence supports first-line treatment with methotrexate and oral glucocorticoids followed by phototherapy and mycophenolate mofetil.

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History

Figure 1	Figure 2

A 65-year-old man initially presented to the Dermatology Clinic at the Veterans Administration New York Harbor Health Care System, Manhattan Campus with an eight- month history of a hypopigmented, linear plaque on his forehead. The lesion extended from his hairline to the mid-forehead, with an additional, smaller, isolated lesion adjacent to his left eyebrow. He reported occasional pruritus at the site but was otherwise asymptomatic. He reported applying Aquaphor daily to the area with no improvement. There were no lesions elsewhere on his body and he denied any antecedent trauma to the area. The patient denied a personal or family history of connective tissue or autoimmune diseases. He denied headaches, vision changes, and history of seizures. The patient initially was treated with doxycycline 100 mg twice daily as well as with clobetasol ointment, but doxycycline was stopped after one month owing to gastrointestinal side effects.

Physical examination

A hypopigmented, indurated, and atrophic linear plaque was located on the left paramedian area of the forehead. It extended approximately 4 cm from the hairline to the mid-forehead. A second, smaller, isolated hypopigmented, indurated, and atrophic plaque was located inferior to the linear plaque on the left glabella.

Laboratory data

A complete blood count was normal. Anti-nuclear antibody, anti-Ro/SSA, and anti-La/SSB titers were negative. Serum protein electrophoresis was normal.

Histopathology

Figure 3	Figure 4

There is a sparse perivascular lymphocytic infiltrate with rare plasma cells and a hypocellular, sclerotic dermis.

Discussion

Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. Morphea has an estimated annual population incidence of 2.7 per 100,000, although this incidence has been increasing slightly since the 1960s. Morphea is divided into subgroups that are based on clinical appearance and distribution. Plaque morphea is the most common form with approximately 56 percent of patients in this category. Linear morphea occurs in 20 percent of patients, whereas generalized and deep morphea affect 13 and 11 percent, respectively [1].

ECDS is a variant of linear morphea with a specific distribution on the frontal aspects of the scalp. Morphea has a slight female predominance that appears to be true for ECDS. ECDS usually presents in childhood, with a median age at onset of approximately ten years, although adult cases exist [2, 3].

ECDS initially appears as a linear, erythematous streak that progresses to an indurated plaque, which may have a surrounding distinct violaceous ring in early lesions. It is typically unilateral and most commonly located in a paramedian distribution on the forehead, but bilateral cases have been reported. It may extend from the frontal hairline downward on the face. Lesions are typically single, but multiple lesions may occur [2]. Several case reports suggest that ECDS may also follow a dermatomal or Blaschkoid pattern [4]. Morphea is thought to be a largely self-limited disease, with an active phase that lasts approximately three to five years followed by subsequent slow resolution of the sclerosis, although the course is variable.

Parry-Romberg syndrome (progressive facial hemiatrophy) may have cutaneous manifestations that are similar to those observed in ECDS. However, it typically displays little-to-no cutaneous sclerosis. These entities may coexist in 20 to 37 percent of patients, which is higher than that observed with other variants of morphea. The relationship between ECDS and Parry-Romberg syndrome is controversial. Some authors advocate a common pathogenesis and believe that these entities exist on a clinical spectrum whereas others believe that they are separate and distinct conditions [2, 3, 5, 6].

In contrast to plaque and generalized morphea but similar to Parry Romberg syndrome, ECDS lesions may involve underlying muscle and bone. Occasionally sclerosis can progress to involve the meninges and brain parenchyma, which leads to neurologic sequelae. In patients with ECDS and/or Parry-Romberg syndrome, seizures are the most commonly reported neurologic manifestation and occur in 73 percent of patients with neurologic symptoms. Focal neurologic deficits, in contrast, are relatively rare. A gadolinium-enhanced magnetic resonance imaging study has been recommended for all patients with ECDS and neurologic symptoms [5]. Ophthalmologic findings also may exist. Extracutaneous findings appear to occur more frequently in children [7].

The etiology of ECDS is unclear, although a growing body of evidence suggests an autoimmune origin, which possibly is initiated by an environmental trigger such as local trauma to the skin. A large percentage of patients with ECDS have elevated titers of one or more autoantibodies, most commonly anti-nuclear antibody (ANA) and anti-single stranded DNA (ssDNA) antibody. Less commonly, rheumatoid factor, anti-histone antibodies, antiphospholipid antibodies, anti-Fcγ receptor antibody, and anti-topoisomerase IIα antibody are elevated, but these occur more typically in generalized morphea. No autoantibody appears to correlate with disease activity [7, 8].

Whether or not Borrelia burgdorferi plays a role in the pathogenesis of morphea is controversial. Some authors believe that early morphea lesions mimic the morphology of erythema chronicum migrans, whereas late-stage lesions are similar to achrodermatitis chronica atrophicans. Furthermore, there are case reports of morphea that responds to antimicrobials, such as antimalarials and penicillin [9]. Although several studies have reported a positive association between Borrelia infection and morphea, numerous others have found no evidence of Borrelia infection in morphea lesions. One reason for this may be the lack of sensitivity for Borrelia detection with current molecular techniques. Another theory is that only certain species, such as Borrelia garinii or Borrelia afzelii, are capable of inducing morphea. To date, however, no large study has found confirmatory evidence of Borrelia species in morphea lesions [10, 11].

Multiple treatments have been proposed for morphea. Evidence suggests that early intervention during the active phase of lesions provides the most benefit and that the treatment modality should in part be determined by lesion depth. For ECDS and linear morphea, a literature review recommends the initial use of methotrexate and systemic glucocorticoids, followed by UVA1, PUVA photochemotherapy, narrow-band UVB phototherapy [12], or mycophenolate mofetil [9]. Other treatment options supported by randomized or prospective trials include topical tacrolimus, topical vitamin D analogs in combination with topical glucocorticoids, and imiquimod [13].

References

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2. 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 [PubMed]

3. Orozco-Covarrubias L, et al. Scleroderma “en coup de sabre” and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 2002; 16:361 [PubMed]

4. Soma Y, Fijimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko’s lines. J Am Acad Dermatol 1998; 38: 366 [PubMed]

5. Kister I, et al. Neurologic manifestations of localized scleroderma: a case report and review of the literature. Neurology 2008; 71: 1538 [PubMed]

6. Jun JH, et al. Parry-Romberg syndrome with en coup de sabre. Ann Dermatol 2011; 23: 342 [PubMed]

7. Marzano AV, et al. Localized scleroderma in adults and children: clinical and laboratory investigations on 239 cases. Eur J Dermatol 2003; 13: 171 [PubMed]

8. Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology 2005; 44: 274 [PubMed]

9. Fett N, Werth VP. Update on morphea: part I and II. Outcome measures and treatment. J Am Acad Dermatol 2011; 64: 231 [PubMed]

10. Eisendle K, et al. Morphoea: a manifestation of infection with Borrelia species? Br J Dermatol 2007; 157: 1189 [PubMed]

11. Weide B, et al. Is morphea caused by Borrelia burgdoferi? Br J Dermatol 2000; 142: 636 [PubMed]

12. Brownell I, et al. Familial linear scleroderma (en coup de Sabre) response to antimalarials and narrow band ultraviolet B therapy. Dermatol Online J 2007; 13:11 [PubMed]

13. Zwischenberger BA, et al. A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol 2011; 65: 925 [PubMed]

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