Neurologic Involvement in Scleroderma en Coup de Sabre

Table 1

Localized scleroderma classification.

Classification	Subtypes	Characteristic lesions	Tissues involved	Main Site
Circumscribed	Superficial variant	Oval lesions	Limited to epidermis and dermis	Trunk
morphea	Deep variant	Oval lesions	Deep indurations. Dermis and subcutaneous tissue involved. Variable muscle and fascia involvement	Trunk
Linear morphea	Trunk/limb variant	Linear indurations	Dermis and subcutaneous tissue (may involve muscle and bone)	Trunk/limb
(linear scleroderma)	Head variant; (en coup de sabre)	Linear indurations	Dermis of the frontoparietal area (may involve muscle, bone, and central nervous system)	Face and scalp
	Parry-Romberg syndrome		Dermis, subcutaneous tissue, muscle, cartilage, and bone	Unilateral face
Generalized morphea		Four or more indurated plaques >3 cm each	Usually limited to the dermis and rarely involves subcutaneous tissue	Diffuse; (no face and hand)
Pansclerotic morphea		Circumferential involvement	Epidermis, dermis, subcutaneous tissue, muscle and bone	Limbs
Mixed variant morphea			Combination of 2 or more previous subtypes