Scleroderma and coup de sabre L94.1

Variant of linear/ligamentous circumscribed scleroderma with localization on the head (most frequently in the forehead area with involvement of the parietal bone) with varying degrees of growth inhibition of the underlying bone. Mostly paramedian sagittal course, more rarely also in the middle of the forehead. Mostly single, more rarely multiple (see Fig.).

The disease is a rarity in the African population (Makhakhe L et al. 2022)

First manifestation usually in childhood. Average age of onset: 10 years. A first manifestation in early adulthood is rarer.

Mainly localized frontoparietally. Usually unilateral, paramedian, but localized centrally in the forehead area, extending into the hairy scalp. It is unclear whether the linear course follows nerves, vessels or Head's zones.

Line or band-shaped, 1-4 cm wide, single or multiple, initially livid, initially flat, sagittally smooth, tender erythema. Over the course of months, the surface depresses in a trough or channel shape. The epidermis appears smooth and shiny with continuously disappearing follicular structures. The color of the lesion changes in the center to a porcelain-like, whitish-grey. The lesional consistency is clearly increased in the center, firm. The edges usually appear in a light reddish but also brownish color. Very often the linear circumscribed scleroderma crosses the forehead-hairline. Infestation of the capillitium leads to lesional, irreversible "scarring" alopecia. With increasing growth inhibition of the skull, the sclerotic center deepens to form a prominent groove or shallow trough.

Neurological manifestations with polyneuropathies and central nervous symptoms with manifestations in the head area should be excluded or monitored by regular clinical neurological examinations as well as imaging procedures, in particular magnetic resonance imaging (MRI) examinations.

With lateral location: Hemiatrophia faciei progressiva.

If only the capillitium is affected, clinical picture of cicatricial alopecia (DD: alopecia areata, here always follicle detection)

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

See below Scleroderma, circumscribed. Augmentation by means of lipoaugmentation is possible. Autologous fat grafting (Coleman's technique) offers a safe and simple approach for the treatment of linear scleroderma "en coups de sabre". With minimal manipulation of the harvested fat in combination with overcorrection of the defect, clinically satisfactory long-term results can be achieved (Ayoub R et al. (2021). Start treatment early to prevent permanent osseous changes.

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.

Early UVA1 irradiation can reduce the inflammatory component. If sclerosis has already occurred, the skin can become significantly softer under UVA1 therapy.

Preoperative UVA1 therapy of the sclerosed area has proven to be effective.

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.

If sclerosis with alopecia has already occurred, the hairless area can be excised. Only a surgeon familiar with this clinical picture should perform this procedure.

Augmentation by means of lipoaugmentation is also possible. Autologous fat grafting offers a safe and simple approach for the treatment of linear scleroderma "en coup de sabre". With minimal manipulation of the harvested fat in combination with overcorrection of the defect, clinically satisfactory long-term results can be achieved (Ayoub R et al. 2021). The extent to which this technique can prevent permanent osseous changes remains to be seen.

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

In a study of 54 subjects, approximately 30% had a combination of hemiatrophia faciei progressiva and scléroderma en coup de sabre. This suggests that there is a pathogenetic connection between the two diseases.

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3. 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

4. Makhakhe L et al. (2022) En coup de sabre morphea: an uncommon condition in Africa. Dermatol Reports15:9537.

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12. 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