Morphea

醫六CLERK吳易儒⿈黃伊妊

TOPIC

DATE REPORT2014.03.06

CHAPTER 64: MORPHEAFITZPATRICK’S DERMATOLOGY 8ED

Outline of morpheaOccurs 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)

Epidemiology2.7 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 !


After breast reconstruction and irradiation Not Metastatic carcinoma

Morphea lesion

Legend:


Clinical Findings


Lichen Sclerosis overlie on Morphea lesions:



A. Typical active morphea plaque in inflammatory stage, with violaceous border. B. In patient with linear morphea-> Severe atrophy (atrophic stage)

Cutaneous Lesions


A. Isomorphic plaques : bra and waistband areas. B. Symmetric plaques

Generalized morphea


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


(involves deep dermis, subcutaneous, fascia, muscle) Morphea profunda: involved areas have “cobblestone” appearance with subcutaneous atrophy.

Deep Morphea(Morphea profunda)


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

AnswerAns:(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

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Morphea