skip to main content

En Coup de Sabre — SCE Dermatology MCQ

Instant feedback + full explanation. One question, done properly.

HardConnective Tissue & VasculitisEn Coup de SabreSCE Dermatology

A 50-year-old woman presents with morphoea (localised scleroderma) involving the forehead in a linear distribution from the hairline to the eyebrow (en coup de sabre). MRI brain shows underlying white matter changes. What is the relationship between en coup de sabre and Parry-Romberg syndrome?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — En coup de sabre and Parry-Romberg syndrome (progressive hemifacial atrophy) are considered to be on the same clinical spectrum — linear scleroderma affecting deeper tissues of the face

En coup de sabre (linear morphoea of the forehead) and Parry-Romberg syndrome (progressive hemifacial atrophy) are now considered part of the same clinical spectrum of craniofacial linear scleroderma. Both involve progressive atrophy of skin, subcutaneous tissue, muscle, and potentially bone on one side of the face/scalp. En coup de sabre presents as a linear band of sclerosis; Parry-Romberg involves more diffuse hemifacial atrophy without the band-like morphology. Both can be associated with neurological complications (seizures, brain parenchymal changes, cranial nerve involvement). MRI brain is recommended to assess for underlying CNS involvement. Treatment is systemic (Methotrexate + corticosteroids) during the active phase.

Reference: BAD 2018 Morphoea; EULAR/PRES