Scleroderma and coup de sabre L94.1

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 08.03.2023

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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Variant of linear/banded circumskriptenous scleroderma of the head (most commonly in the frontal region involving the parietal ossa) 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.

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Dermatology

Skin nerve

Small peripheral nerves supplying the skin, arranged segmentally; see also under dermatome.

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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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Circumscribed scleroderma.

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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In case of lateral seat: Hemiatrophia faciei progressiva. In case of exclusive affection of the capillitium, clinical picture of scarring alopecia (DD: alopecia areata, here always follicle detection).

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 acute inflammatory stage, topical glucocorticoids under occlusion may be used for a short time. Alternatively, topical calcineurin inhibitors (e.g., tacrolimus, pimecrolimus) can be used (strictest indication because of unclear long-term side effects!).

In the sclerotic stage, topical therapy with vitamin _D3 analogues may induce additional softening of the focus. Similar to UVA1 therapy, vitamin _D3 analogues lead to the induction of collagenase in 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 sclerosis with alopecia has already occurred, excision of the hairless area can be performed. Here, only the scleroderma-experienced surgeon should act.

If severe and deforming osseous deformity has occurred, appropriate elevation of the skull roof and, if necessary, reconstruction of the forehead or orbit are possible in specialized centers.

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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Dirschka T et al (2007) Surgical correction of scleroderma en coup de sabre by en bloc resection. Dermatologist 58: 611-614
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
Marzano AV et al (2003) Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 13: 171-176.
Mertens JS et al. (2015) Disease recurrence in localized scleroderma: a
retrospective analysis of 344 patients with paediatric- or adult-onset disease.
Br J Dermatol 172:722-728
Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma en coup de sabre. Dermatologist 45: 398-401
Polcari I et al (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. Pediatrics 134: e1715-1719 .
Rai R et al (2000) Bilateral en coup de sabre-a rare entity. Pediatr Dermatol 17: 222-224.
Sehgal VN et al (2002) En coup de sabre. Int J Dermatol 41: 504-505.
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.