Morphea (linear localized scleroderma)

Diagnosis: Morphea (linear localized scleroderma)

Linear atrophic depression of an en coup de sabre lesion on the right side of the forehead and the frontal part of the scalp.

Clinical Presentation

Linear atrophic depression of an en coup de sabre lesion on the right side of the forehead and the frontal part of the scalp.

Clinical History

Submitted by Alaa Saad. Originally posted September 11, 2010.

Treatment

See case discussion.

Differential Diagnosis

• Systemic sclerosis (scleroderma) • Lichen sclerosus • Radiation dermatitis • Nephrogenic systemic fibrosis • Eosinophilic fasciitis • Vitiligo (early morphea) • Lipodermatosclerosis

Key Learnings

• Localized scleroderma — fibrosis confined to skin ± subcutaneous tissue • Does NOT typically progress to systemic sclerosis (different disease) • "Lilac ring" sign: violaceous border indicates active, inflammatory phase • En coup de sabre: linear morphea of the forehead — Parry-Romberg syndrome overlap • Histology: thickened, homogenized collagen bundles replacing subcutaneous fat • Active disease: positive anti-ssDNA, elevated eosinophils, elevated ESR • Linear morphea in children can affect bone growth if crossing joints

Tags: morphea, linear, localized, scleroderma, alaa saad