Morphea, also known as localized scleroderma, is an inflammatory skin condition that can cause severe functional and cosmetic damage. A rare disease with an unknown etiology, it increases collagen deposition and, consequently, skin thickness in adults. Both men and women are affected, with a 3:1 female predominance.1,2
Morphea is divided into four subtypes: linear, generalized, plaque (circumscribed), and mixed. Linear morphea, as the most common subtype in children, is often associated with musculoskeletal, cosmetic, and neurologic irregularities.
Linear morphea is distinguished by sclerotic skin lesions distributed in a linear, band-like pattern.3 It generally appears as an atrophic sclerotic band over the frontoparietal region, with varying degrees of skin discoloration, during the first or second decade of life. In 67% of the cases, linear morphea is detected before age 18.4 Although bilateral occurrences and cases involving the lower face have been documented, the lesions are often unilateral, extending no lower than the eyebrow. Because these skin lesions resemble the stroke of a sword, the condition is referred to as scleroderma en coup de sabre.5 Differential diagnosis includes post-traumatic scarring, facial atrophy known as Parry-Romberg syndrome, and linear scleroderma.6
The linear morphea subtype is usually characterized by deep involvement, with a risk of functional limitations that may warrant more aggressive treatment. Appropriate therapy will reduce sclerotic activity and stabilize the damage. Common modalities include topical steroids and vitamin D derivatives; a combination of methotrexate and corticosteroids may be helpful during the inflammatory stage of progressive linear morphea.7
Because linear morphea may be triggered by localized trauma to the skin, dental clinicians should be aware of the risks when diagnosing any case.8 A pediatric dentist may be the first medical professional to notice linear morphea because of its involvement with the dentition.9 Orthodontic treatment will often involve correction of facial asymmetry related to the soft or hard tissues, as demonstrated in the following case report.
Diagnosis and Treatment Planning
A 20-year-old female presented to the orthodontic department with the chief complaint of spacing in the maxillary anterior region. Clinical examination found a mesocephalic head shape, a mesoprosopic facial form with competent lips and posterior facial divergence, and an unesthetic smile (Fig. 1A). The patient had bilateral Class I molar relationships, 2mm of overjet, 3mm of overbite, spacing in both arches, and a crossbite of the lower left first molar.
Fig. 1 20-year-old female patient with hypodivergent growth pattern, unilateral crossbite, spacing in both arches, and facial asymmetry attributable to linear morphea before treatment (continued in next image).
Cephalometric analysis (Table 1) indicated a skeletal Class I malocclusion (ANB = 1°) with a hypodivergent growth pattern (SN-GoMe = 30°), proclined upper incisors (U1-NA = 37°, 8mm), and retroclined lower incisors (L1-NB = 16°, 4mm) (Fig. 1B).
Fig. 1 (cont.) 20-year-old female patient with hypodivergent growth pattern, unilateral crossbite, spacing in both arches, and facial asymmetry attributable to linear morphea before treatment.
Facial asymmetry due to a lesion was evident in the right submandibular area. The patient reported no medical history relating to the asymmetry and instead gave a history of trauma. Since we doubted that the patient was being truthful about the lesion, she was referred to the department of dermatology for a skin biopsy, which revealed a focal hyperkeratosis with dense fibrosis. Considering the clinical findings, the specialists confirmed the lesion to be linear morphea. At that point, the patient admitted that she had been undergoing treatment for linear morphea for seven years, but that she was afraid it might affect her chances of having orthodontic treatment. Autogenous fat grafts had been performed three years earlier by a team of plastic surgeons, achieving an overcorrection of 20%, but the disease had started to progress again only one year later.
Treatment objectives were to correct the crossbite of the lower left first molar, correct the incisor inclinations, close extraneous spaces, maintain bilateral Class I canine and molar relationships, and provide an esthetic soft-tissue profile with optimal overjet and overbite, while minimizing orthodontic intervention to avoid any soft-tissue laceration that could aggravate the linear morphea.
Conventional treatment would have involved surgical management of the facial asymmetry using autogenous fat grafts, cartilage grafts, or silicone injections, followed by orthodontic therapy. Because the patient was not yet ready for a second round of autogenous grafting, however, and any kind of trauma during surgery could have exacerbated the linear morphea,8 we decided to proceed with nonextraction orthodontic treatment only.
Treatment Progress
To correct the posterior crossbite, a lower lingual holding arch was fabricated from blue Elgiloy* wire (Fig. 2). The holding arch was constricted by 2mm; buccal crown torque was added at the lower right first molar to anchor unilateral constriction of the lower left first molar, which was outside the catenary curve.
Fig. 2 Lower lingual holding arch fabricated from blue Elgiloy* wire for correction of posterior crossbite; holding arch constricted by 2mm, and buccal crown torque added at lower right first molar to anchor unilateral constriction of lower left first molar.
After four months, when the crossbite was substantially corrected, both arches were bonded with .022" × .028" MBT**-prescription edgewise brackets. Leveling and alignment were initiated with .014" nickel titanium archwires and completed with .016" × .022" nickel titanium archwires, followed by .017" × .025" and .019" × .025" stainless steel archwires for correction of the anterior tooth inclinations. The upper and lower anterior spaces were closed using continuous elastomeric chain along with the final archwires.
After five months of fixed orthodontic therapy, the brackets were debonded and fixed upper 1-1 and lower 4-4 lingual retainers were delivered. Since the crown width-to-height ratios of the right and left lateral incisors were less than .8, the patient was referred to the department of conservative dentistry for composite build-ups to provide an esthetic finish.10
Treatment Results
Total treatment time was nine months (Fig. 3A). The patient exhibited an improved lip seal and properly inclined maxillary and mandibular incisors (Table 1). Class I canine and molar relationships with adequate overjet and overbite, coincident midlines, and a more esthetic smile were achieved. Final panoramic radiographs confirmed good root parallelism (Fig. 3B).
Fig. 3 A. Patient after nine months of treatment (continued in next image).
Fig. 3 (cont.) A. Patient after nine months of treatment. B. Superimposition of pre- and post-treatment cephalometric tracings.
Discussion
Scleroderma en coup de sabre, first described by Addison in 1854, is unique because it affects only half the face and progresses slowly but steadily.11 Linear morphea may affect the skin, adipose tissue, fascia, muscle, and bone. The deeper layer of the connective tissue becomes affected with increasing severity of the fibrosis.12 Dental, skeletal, and soft-tissue pathology may combine to create facial asymmetry, as in the case shown here.
There are few reports on patients with linear morphea in the orthodontic literature. Our case is noteworthy because we were able to achieve a favorable result with minimal orthodontic treatment and no surgical intervention.
FOOTNOTES
- *Registered trademark of Rocky Mountain Orthodontics, Denver, CO; www.rmortho.com.
- **Unitek, trademark of 3M, Monrovia, CA; www.3M.com.
REFERENCES
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- 2. Cho, H.K. and Chun, S.I.: A clinical study of localized scleroderma, Kor. J. Dermatol. 34:109-115, 1996.
- 3. Kunzler, E.; Florez-Pollack, S.; Teske, N.; O’Brien, J.; Prasad, S.; and Jacobe, H.: Linear morphea: Clinical characteristics, disease course, and treatment of the morphea in adults and children cohort, J. Am. Acad. Dermatol. 80:1664-1670, 2019.
- 4. Careta, M.F. and Romiti, R.: Localized scleroderma: Clinical spectrum and therapeutic update, An. Bras. Dermatol. 90:62-67, 2015.
- 5. Itin, P.H. and Schiller, P.: Double-lined frontoparietal scleroderma en coup de sabre, Dermatol. 199:185-186, 1999.
- 6. Kim, J.H.; Lee, S.C.; Kim, C.H.; and Kim, B.J.: Facial asymmetry: A case report of localized linear scleroderma patient with muscular strain and spasm, Maxillofac. Plast. Reconstr. Surg. 37:29, 2015.
- 7. Docrat, M.E.: Morphea (localised scleroderma): Skin focus, Curr. Allergy Clin. Immunol. 19:192-194, 2006.
- 8. Grabell, D.; Hsieh, C.; Andrew, R.; Martires, K.; Kim, A.; Vasquez, R.; and Jacobe, H.: The role of skin trauma in the distribution of morphea lesions: A cross-sectional survey of the morphea in adults and children cohort IV, J. Am. Acad. Dermatol. 71:493-488, 2014.
- 9. Hørberg, M.; Lauesen, S.R.; Daugaard-Jensen, J.; and Kjær, I.: Linear scleroderma en coup de sabre including abnormal dental development, Eur. Arch. Paediat. Dent. 16:227-231, 2015.
- 10. Álvarez-Álvarez, L.; Orozco-Varo, A.; Arroyo-Cruz, G.; and Jiménez-Castellanos, E.: Width/length ratio in maxillary anterior teeth: Comparative study of esthetic preferences among professionals and laypersons, J. Prosthod. 28:416-420, 2019.
- 11. Chiang, K.L.; Chang, K.P.; Wong, T.T.; and Hsu, T.R.: Linear scleroderma “en coup de sabre”: Initial presentation as intractable partial seizures in a child, Pediat. Neonatol. 50:294-298, 2009.
- 12. Fox, T.C.: Note on the history of scleroderma in England, Br. Dermatol. 4:101-104, 1892.
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DR. KUMAR -
DR. GOYAL -
DR. AGARWAL -
DR. MISHRA -
DR. BOHARA
Dr. Kumar is a Professor, Dr. Goyal is a Professor and Head of Department, and Dr. Mishra is a Senior Lecturer, Department of Orthodontics and Dentofacial Orthopaedics, Teerthanker Mahaveer Dental College, Moradabad, 244001 Uttar Pradesh, India. Dr. Agarwal is a Consultant Orthodontist, Dental Vaidya, Jharkhand, India. Dr. Bohara is a Consultant Orthodontist, Dr. Bohara’s Vardhman Dental Clinic, Camp, Pune, Maharashtra, India. E-mail Dr. Kumar at drmukeshortho@yahoo.co.in.
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