Dermatology · Connective Tissue Disorders in Skin (Lupus, Scleroderma)

A 12-year-old girl has a band of ivory-white, bound-down skin extending from the scalp across the forehead, with loss of hair in the affected area and atrophy of the underlying facial tissues on that side. The most likely diagnosis is:

  • A Linear morphea of the en coup de sabre type
  • B Discoid lupus erythematosus
  • C Generalised morphea
  • D Necrobiosis lipoidica
Correct answer: A. Linear morphea of the en coup de sabre type

Explanation

En coup de sabre is linear morphea of the frontoparietal scalp and face, producing a depressed scar-like band with cicatricial alopecia. When it extends to involve hemifacial soft tissue and bone atrophy, it merges with Parry-Romberg progressive hemifacial atrophy. Discoid lupus gives scaly inflammatory plaques rather than a linear bound-down band, generalised morphea is bilateral and truncal, and necrobiosis lipoidica is an annular shin lesion of diabetes.

Reference: Rook's Textbook of Dermatology, 9th ed.

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