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Skin&soft tissue manifestation in autoimmune disease (SLE…
Skin&soft tissue manifestation in autoimmune disease
SLE
Concept
Multi-organ-Ab autoimmune diseases
Female > male
Pathogenesis
Loss tolerance
Autoantibodies
Anti-dsDNA
Specific diagnosis
Nephritis
Anti-Sm
Specific diagnosis
Nucleosome
Leading cell abnormal apoptosis
Histone structure
dsDNA
Ag of SLE
Genetic
In monozygtic twins
Hormone
Estrogen
Immune
Innate
TLR9 : DNA
TLR7 : RNA
Adaptive
HLA II
DR, DP, DQ
Complement def.
C1, C2, C4
Low serum complement
C3, C4, CH50 ต่ำ (classical pathway)
Cytokine ต่ำ
CRP ต่ำ
C5b-9
negative
Type III
hypersensitivity
Criteria
มากกว่าเท่ากับ 4 Criteria (at least 1 clinical and 1 immunologic)
Clinical appearance
Clinical
Skin
LE-Specific skin disease
Acute cutaneous LE
Malar rash
Erythematous rash over cheeks and nasolabial folds (butterfly-shaped)
IgG deposit at capillary of DEJ
Systemic disease
Chronic cutaneous LE
Skin disease
Discoid rash
Oral lesion
1 more item...
Keratotic scaling, follicular pluging
Hypopigment
IgM deposit at dermis
Lupus panniculitis หลุมที่ skin
Subacute cutaneous LE
Skin lesion
Annular
Papulosquamous
Photosense
ด้านในแขนไม่เกิด annular lesion
LE non specific skin disease
Vasculitis
Palpable purpura (กดแล้วเลือดไม่จาง)
Systemic disease
Serositis
Arthritis
Pleuritis
Pericarditis
Renal
24h urine protein >0.5 g
Hemato
Pancytopenia
Neurologic
Immunologic
Disease
DLE
ANA 30%
SCLE
ANA 80%
SSa/Ro 70%
SLE
ANA 98%
SSa/Ro 40%
dsDNA 30%
ANA
Lupus band test
Positive
: IgG
without skin lesion
Acute lesion :
positive
Chronic lesion :
negative
Treatment
Reduce inflammation
Steroids
Remove CIC
Plasmapheresis : severe มาก
Inhibit proliferation of T and B cell
Mycophenolate, mofetil, azathioprine, cyclophsphamide
Dermatomyositis
Clinical appearance
Muscle
Symmetric proximal muscle weakness :warning: common
CKMB
EMG and muscle biopsy
Skin
Grotton’s sign
Erythematousover metacarpophalangeal joints
Heliotrope rash
:warning:
Most specific
purple rash at upper eyelid
Calcinosis cutis
Calcium deposit
Generalized erythroderma
Shawl sign and V sign : rash ขึ้นแนวผ้าคลุมไหล่, คอเสื้อ
Pruritic skin
Mechanic hand
Capillary loop dilation, periungual erythema
Photosensitive
Lab
Auto Ag
Anti-MDA5
Dermatomyositis
Anti-Mi-2
เฉพาะ dermato
Lupus band test
usually
negative
C5b-9
Positive
deposit at DEJ
No hypo
complementemia
Treatment
Improve muscle strength
Glucocorticoids
Not return to normal strength
Pathogenesis
Type III
hypersensitivity
CIC deposit and activate in vss.
Rash and myositis
Female > male
Scleroderma
Pathogenesis
Abnormal blood vss
เริ่มมาก่อน มี Reynaud phenomenon
Chronic ischemia, over fibrosis
Autoimmunity
Classification
Localized scleroderma
Linear scleroderma
Depressed scar, color change
Systemic sclerosis
Limited cutaneous
Skin thickening (เว้นเหนือศอกกับเข่า)
Anti-Centromere Ab
Prognosis ดี
Disease progress after
Raynaud phenomenon
years
Complication
Pulmonary hypertension
Late internal organ
Diffuse cutaneous
Skin thickening area above elbow and knee (เป็นทั้งตัว)
Disease Rapid onset after
Raynaud phenomenon
month
Early internal organ involvement
Anti-Scl-70
Prognosis ไม่ดี
Complication
Pulmonary fibrosis
Scleroderma renal crisis
Abnormal healing
Over fibrosis responses
High TGF-beta
Clinical appearance
Skin
Face
Microstomia (อ้าปากกว้างไม่ได้)
Dental disease (ปิดปากไม่ได้)
Loss of nasolabial fold
Salt and pepper skin
Perifollicular sparing of pigment
Digital ulcer
3 phases
Lymph fibrosis มือไม่แดง เริ่มมือแข็ง joint แข็ง
Nail fold change
VEGF
Chronic hypoxia, ischemia
Calcinosis
Calcium deposit under skin
Sign of damage
Internal organ involvement
Pulmonary fibrosis
Pericardial fibrosis
Myopathy
Scleroderma renal crisis
Hypertension due to
renal ischemia
Treat by ACEI (vasodilator)
Grave prognosis !!!
Supportive treatment