2. Outline of morphea
Occurs in children and adults
Children: Linear subtype
Adults: Circumscribed and generalized subtypes
A self-limited or chronically relapsing autoimmune disorder
Inflammatory, sclerotic, atrophic
Thickened sclerotic skin
Systemic disease: arthritis, neurological disorders
Differentiated from scleroderma(localized)
3.
4.
5. Epidemiology
2.7 per 100,000
female:male=2:3.1
20-30% begin in childhood(can occur any age)
25-80% of pediatric subtype are linear
70-80% limb/trunk
22-30% ECDS(en coup de sabre)/PHA(Progressive
facial hemiatrophy)
3-6 years, 20% reactivation
15. A. Multiple linear lesions involving trunk and extremities.
B. B. En coup de sabre.
C. Multiple hyperpigmented linear morphea lesions on the face.
Linear Morphea
18. squared-off edge of the biopsy specimen with
mild superficial and deep inflammatory infiltrate and
broadened compact collagen fibers, generally parallel to the epidermis.
Histopathology
19. Q & A
What may elevate when we check Morphea
patients' specimen?!
!
(A) ANA!
(B) IFN-α!
(C) IL-10!
(D) None of above
20. Answer
Ans:(A)!
Morphea is related to familial autoimmune disorders.!
!
Autoantibodies reported include:!
ANA(39-80%), antisincle-stranded DNA, antidouble-
stranded DNA, antihistone, antitopoisomerase IIα,
antiphospholipid, anticentromere, anti-Scl-70!
!
And reumatic factor:!
MMP-1