Connective  tissue disorders
nLupus erythematosus
nScleroderma
nDermatomyositis, polymyositis
nOverlap syndromes

Lupus erythematosus
nsystemic lupus erythematosus   -  SLE
nchronic cutaneous lupus erythematosus – CCLE
nsubacute cutaneous lupus erythematosus – SCLE
nneonatal LE
ndrug induced ( hydralazine, sulfonamides)
n
n

Systemic lupus erythematosus   -  SLE
nARA criteria:
nmalar rash
ndiscoid lesions
nphotosensitivity
noral lesions
narthritis non-erosive
nserositis
nneurologic disorders
nlupus nephritis ( proteinuria 0,5g/d)
nhematologic disorders
nimmunologic disorders ( LE cells, ANA         , anti dsDNA, Sm antigen, histones etc.)
ANAhom

http://image.slidesharecdn.com/vaishali-140929111755-phpapp01/95/case-of-sle-10-638.jpg?cb=14119896
39
(SLICC - SLE international cooperating clinics group)

sle-cde-oblicej sle-motylek-muz Obsah obrázku interiér Popis byl vytvořen automaticky



SLE
gangrena-diabeticorum kytlicova20080526_0008 128-2850_IMG


Chronic cutaneous lupus erythematosus - CCLE
ndiscoid lesions – CDE –scarring -lupus
nphotosensitivity – sun exposed areas
nhypertrophic lesions –lupus tumidus - rare
nlupus panniculitis
nANA low titers or none (≤1:160)
nno systemic disease
n
nsymptom of systemic disease (30%)
n

122-2298_IMG cde-oblicej cde-oblicej-zena cde-oblicej-muz Obsah obrázku osoba, muž, tetování,
sousto Popis byl vytvořen automaticky Obsah obrázku osoba, žena, růžová, vlasy Popis byl vytvořen
automaticky

Lupus panniculitis
n
IMG_5655 tomanova

CDE – oral and lip involvement
n
6-131 6-14

Subacute cutaneous lupus erythematosus - SCLE
nannular lesions
npapulosquamous lesions
nphotosensitivity
nsicca syndrome secondary
nANA, anti Ro/ SSA, La/SSB
nmild systemic disease
nneonatal LE  (Ro 52 kD)

scle-trup-celek-zena 110-1079_IMG
xxx
xxx

n
henčlova 20080401_0015
20071109_0003

Lupus erythematosus
nTherapy:
nantimalarial drugs (hydroxychlorochine)
ncorticosteroids
nimmunosuppresives (cyclophosphamide, cyclosporine, MTX, azathioprine)
nantiinflammatory drugs
nUVA, UVB sunscreens
nplasmapheresis, pulse therapy etc.
nBelimumab (BAFF)

Scleroderma
nvascular changes – fibroplastic factors activation
n
nchanges in collagen synthesis
n
nimmunological changes –
n   humoral   (anti Scl70, ANA   , anti centromere ACA)
n   cellular
Plné kosočtverce




Systemic sclerosis - scleroderma
nARA criteria:
nproximal scleroderma
nbilateral lung interstitial fibrosis
nfingertip changes DUC digital ulcerations
nsclerodactyly
nother signs:
nRaynaud ´s phenomenon
nesophageal changes
nrenal disease
npulmonary hypertension
npericardial effusion

sclerodermia-diffusa-sklerodatylie sclerodermia-diffusa-obl sklerodermie-difusni-oblicej-zena-u
akrocyanoza-ss


Scleroderma
153-5382_IMG 153-5384_IMG 153-5386_IMG


CREST syndrome – limited scleroderma
nC alcinosis
nR aynaud
nE sophageal changes
nS cleroderma
nT eleangiectasias
nanti centromere antibody

Systemic scleroderma
nTherapy:
n corticosteroids
n immunosuppresives
nantimalarials
nd-penicillamine, penicillin
nvasoactive and rheological drugs (pentoxiphylline, prostavasin)
ncalcium antagonists – nifedipine
nendothelin receptor antagonist – bosentan
nphosphodiesterase 5 antagonists - sildenafil
nACE inhibitors - kidneys
nprokinetic drugs, antacids
nphysiotherapy

Localized scleroderma
nmorphea
nguttate
nlinear
ngeneralized (pansclerotic morphea)
nsubcutaneous – eosinophilic fasciitis
nANA in low titers or none
nno systemic disease
ninfections – late stage Lyme disease

morfea-bok-zena morfea-detail-lilac-ring morphea-kriz sklerodermie-linearni-stehno
Lilac ring


Localized scleroderma
n
120-2018_IMG I~000054

Linear scleroderma
sklerodermia-jazyk-tvar-dite nik3788 sklerodermie-linearni-stehno
XX

Localized scleroderma - therapy
nTopical – corticosteroids, deltanoids, heparin
nSystemic- PNC
nCorticosteroids
nMTX
nAntimalarials
nUVA1

Dermatomyositis, polymyositis
njuvenile - association with infections
nadult  - association with tumours (paraneoplastic)
nheliotrophic rash, Gottron´s sign, poikilodermatitis, erythemas
nEMG
nANA, anti Jo-1
nCPK, LDH, GOT, ALD, AST, ALT, myoglobin
nHistology – muscle, skin

dmkrut-celo dmkrut-hrud dmkrut-ruce dmkrut-prsty



n
foto klinika 06 07 014


118-1847_IMG 118-1821_IMG



Dermatomyositis - therapy
nCorticosteroids
nImmunosuppresives - MTX, azathioprine, cyclosporine
nAntimalarials
nIVIG