Morphea (localized scleroderma)

Diagnosis: Morphea (localized scleroderma)

Inactive disease with atrophy mottled hypo and hyperpigmentation.

Clinical Presentation

Inactive disease with atrophy mottled hypo and hyperpigmentation.

Clinical History

Submitted by Alaa Saad. Originally posted September 18, 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, localized, scleroderma, alaa saad