Scleroderma and coup de sabre L94.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

en coup des sabre scleroderma; linear morphea and coup de sabre; linear scleroderma and coup de sabre; Sabre cut scleroderma; Scleroderma and coup de sabre; scleroderma en coup de sabre

Definition
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Special form of linear/band-shaped circumscribed scleroderma of the head (most frequently in the forehead area) with varying degrees of growth inhibition of the underlying bone,

Manifestation
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Preference is given to a pediatric clientele with a mean age of 10 years. The first manifestation in early adulthood is rarer.

Localization
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Mainly frontoparietal location. Mostly unilateral, paramedian of the eyebrows extending into the hairy scalp.

Clinical features
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Initial livid erythema, then development of a whitish sclerosis in the centre. As the skull grows, the sclerotic zone sinks with pronounced furrow formation.

Histology
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Diagnosis
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Characteristic clinical picture. There should be a neurological examination and an MRI of the skull to exclude CNS involvement.

Differential diagnosis
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For lateral seat: Hemiatrophia faciei progressiva. If the capillitium is exclusively affected, clinical picture of scarring alopecia (DD: Alopecia areata, here always follicle evidence)

Complication(s)
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Neurological complications: headache, migraine. These complaints can precede the cutaneous changes for several months.

Therapy
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S.u. Scleroderma, circumscript. In the case of burnt-out forms, a build-up by means of lip augmentation is possible. Early start of therapy to prevent permanent osseous changes.

External therapy
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  • In the acutely inflammatory stage, if necessary short-term topical glucocorticoids under occlusion. Alternatively, topical calcineurin inhibitors (e.g. Tacrolimus, Pimecrolimus) can be applied (strictest indication because of unclear long-term side effects!).
  • In the sclerotic stage, topical therapy with vitamin D3 analogues may induce an additional softening of the focus. Similar to UVA1 therapy, vitamin D3 analogues lead to the induction of collagenase in the fibroblasts.

Radiation therapy
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Early UVA1 irradiation can reduce the inflammatory component. If sclerotherapy has already occurred, the skin may soften under UVA1 therapy. UVA1 irradiation has proven to be effective before surgical repair of sclerotic plaque.

Internal therapy
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In acute phases, MTX can be used in combination with systemic glucocorticoids for several months in addition to external therapy and UVA1 irradiation. The indication must always be determined individually.

Operative therapie
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  • If sclerotherapy with alopecia has already occurred, the excision of the hairless area can be performed. In this case only the surgeon experienced in scleroderma should be used.
  • If an osseous deformation has occurred, the skull roof can be raised accordingly and, if necessary, the forehead or eye socket can be reconstructed in specialised centres.

Progression/forecast
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Good prognosis with early therapy. Sclerotherapy that has already occurred usually results in permanent notching with varying degrees of facial disfigurement.

Note(s)
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In a study with 54 volunteers, about 30% had hemiatrophia faciei progressiva and scléroderma en coup de sabre simultaneously. This suggests that there is a pathogenetic connection between both diseases.

Literature
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  1. Dirschka T et al (2007) Operative correction of scleroderma and coup de sabre by en-bloc resection. Dermatologist 58: 611-614
  2. Flores-Alvarado DE et al (2003) Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship? J Rheumatol 30: 193-195
  3. Marzano AV et al (2003) Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 13: 171-176
  4. Mertens JS et al (2015) Disease recurrence in localized scleroderma:
    aretrospective analysis of 344 patients with paediatric- or adult-onset disease.
    Br J Dermatol 172:722-728

  5. Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma and coup de sabre. dermatologist 45: 398-401

  6. Polcari I et al (2014) Headaches as a presenting symptom of linear morphea and coup de sabre. Pediatrics 134: e1715-1719 .
  7. Rai R et al (2000) Bilateral en coup de sabre-a rare entity. Pediatrics Dermatol 17: 222-224
  8. Sehgal VN et al (2002) En coup de sabre. Int J Dermatol 41: 504-505
  9. Tollefson MM et al (2006) En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 56: 257-263

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020