Synonym(s)
DefinitionThis section has been translated automatically.
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.
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.
You might also be interested in
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.
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)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 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 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 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/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) 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.
Outgoing links (7)
Calcineurin inhibitors; Circumscripts of scleroderma (overview); Glucorticosteroids topical; Parry Romberg syndrome; Pimecrolimus; Tacrolimus; Uv rays;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.