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En coup de sabre

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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This is purely cutaneous disorder often classified as a localised form of scleroderma (which is relatively benign and well circumscribed) but dermopathologically distinct (1).

It is an autoimmune condition characterized by excessive collagen deposition leading to thickening of the skin (2).

It usually affects children and young adults whom develop spontaneously, a scar-like band or plaque along the line of a limb or on the scalp - "en coup de sabre".

It is more common in females than in males (2).

Localized scleroderma differs from systemic scleroderma due to the absence of :

  • Raynaud's phenomenon
  • acrosclerosis
  • systemic organ involvement
  • prognosis of the patient is better than in systemic sclerosis (1)

Morphoea can be classified into 5 groups according to the clinical presentation:

  • generalized morphea: is the severe variety of morphoea with extensive involvement of the skin.
  • plaque morphea : may present as multiple small, flat or slightly depressed patches or as flat topped white papules that might join together to form plaques, involvement is limited to the dermis
  • bullous morphea: presents as a tense subepidermal bullae which coexist with typical plaque morphea
  • linear morphea: commonly seen in children and presents on limbs (occasionally on the forehead and rarely on the anterior trunk) as single linear and unilateral bands. It may result in complications such as growth retardation, joint contractures, and muscle atrophy
  • deep morphea: diffuse lesions which involves the deeper tissues e.g. - deep dermis, subcutaneous tissue, fascia, or muscles

Click here for an example image of this condition



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