DefinitionThis section has been translated automatically.
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,
ManifestationThis section has been translated automatically.
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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LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
HistologyThis section has been translated automatically.
DiagnosisThis section has been translated automatically.
Characteristic clinical picture. There should be a neurological examination and an MRI of the skull to exclude CNS involvement.
Differential diagnosisThis section has been translated automatically.
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)This section has been translated automatically.
Neurological complications: headache, migraine. These complaints can precede the cutaneous changes for several months.
TherapyThis section has been translated automatically.
External therapyThis section has been translated automatically.
- 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 therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
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 therapieThis section has been translated automatically.
- 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/forecastThis section has been translated automatically.
Note(s)This section has been translated automatically.
LiteratureThis section has been translated automatically.
- Dirschka T et al (2007) Operative correction of scleroderma and 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:
aretrospective 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 and coup de sabre. dermatologist 45: 398-401
- Polcari I et al (2014) Headaches as a presenting symptom of linear morphea and coup de sabre. Pediatrics 134: e1715-1719 .
- Rai R et al (2000) Bilateral en coup de sabre-a rare entity. Pediatrics 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
Incoming links (1)Hemiatrophia faciei progressiva;
Outgoing links (7)Calcineurin inhibitors; Circumscripts of scleroderma (overview); Glucorticosteroids topical; Hemiatrophia faciei progressiva; Pimecrolimus; Tacrolimus; Uv rays;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.