醫六CLERK吳易儒 ⿈黃伊妊
TOPIC
DATE REPORT
2014.03.06
CHAPTER 64: MORPHEA
FITZPATRICK’S DERMATOLOGY 8ED
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)
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
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
Chronicity and sequelae
left leg, 5y/o
abdomen, 16 y/o
right shoulder, 19 y/o
muscle atrophy, limb length discrepancy, pes plans foot deformity
From: Chapter 64. Morphea
Fitzpatrick's Dermatology in General Medicine, 8e, 2012
!
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
After breast reconstruction and irradiation
Not Metastatic carcinoma
Morphea lesion
Legend:
From: Chapter 64. Morphea
Fitzpatrick's Dermatology in General Medicine, 8e, 2012
!
Clinical Findings
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
Lichen Sclerosis overlie on Morphea lesions
:
From: Chapter 64. Morphea
Fitzpatrick's Dermatology in General Medicine, 8e, 2012
!
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
A. Typical active morphea plaque in inflammatory stage, with violaceous border.
B. In patient with linear morphea-> Severe atrophy (atrophic stage)
Cutaneous Lesions
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
A. Isomorphic plaques : bra and waistband areas.
B. Symmetric plaques
Generalized morphea
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
majority of body surface area (A),
sparing the fingertips (B).
Pansclerotic morphea
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
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
(involves deep dermis, subcutaneous, fascia, muscle)
Morphea profunda: involved areas have “cobblestone” appearance with subcutaneous atrophy.
Deep Morphea(Morphea profunda)
Date of download: 3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved.
Arthritis: swollen right knee with effusion in patient with morphea.
Related Physical Findings
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
Q & A
What may elevate when we check Morphea
patients' specimen?!
!
(A) ANA!
(B) IFN-α!
(C) IL-10!
(D) None of above
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
THANKS

Morphea

  • 1.
  • 2.
    Outline of morphea Occursin 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)
  • 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
  • 6.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. Chronicity and sequelae left leg, 5y/o abdomen, 16 y/o right shoulder, 19 y/o muscle atrophy, limb length discrepancy, pes plans foot deformity From: Chapter 64. Morphea Fitzpatrick's Dermatology in General Medicine, 8e, 2012 !
  • 7.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. After breast reconstruction and irradiation Not Metastatic carcinoma Morphea lesion Legend: From: Chapter 64. Morphea Fitzpatrick's Dermatology in General Medicine, 8e, 2012 !
  • 8.
  • 11.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. Lichen Sclerosis overlie on Morphea lesions : From: Chapter 64. Morphea Fitzpatrick's Dermatology in General Medicine, 8e, 2012 !
  • 12.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. A. Typical active morphea plaque in inflammatory stage, with violaceous border. B. In patient with linear morphea-> Severe atrophy (atrophic stage) Cutaneous Lesions
  • 13.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. A. Isomorphic plaques : bra and waistband areas. B. Symmetric plaques Generalized morphea
  • 14.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. majority of body surface area (A), sparing the fingertips (B). Pansclerotic morphea
  • 15.
    A. Multiple linearlesions involving trunk and extremities. B. B. En coup de sabre. C. Multiple hyperpigmented linear morphea lesions on the face. Linear Morphea
  • 16.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. (involves deep dermis, subcutaneous, fascia, muscle) Morphea profunda: involved areas have “cobblestone” appearance with subcutaneous atrophy. Deep Morphea(Morphea profunda)
  • 17.
    Date of download:3/4/2014 Copyright © 2012 McGraw-Hill Medical. All rights reserved. Arthritis: swollen right knee with effusion in patient with morphea. Related Physical Findings
  • 18.
    squared-off edge ofthe biopsy specimen with mild superficial and deep inflammatory infiltrate and broadened compact collagen fibers, generally parallel to the epidermis. Histopathology
  • 19.
    Q & A Whatmay 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 relatedto 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
  • 21.