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CONNECTIVE TISSUE DISEASES
1.Lupus Erythematosus (LE)2.Dermatomyositis3.Scleroderma4.Rheumatoid5.Sjogren’s Syndrome6.Eosinophilic Fasciitis7.Mixed Connective Tissue Disease (MCTD)8.Relapsing Polychondritis
LUPUS ERYTHEMATOSUSI. CHRONIC CUTANEOUS L.E
.
•
Discoid LE - Generalized- Localized 
•
Lupus Profundus (Lupus panniculitis)
II. Subacute Cutaneous LE
Acute Cutaneous L.E. (Systemic L.E.)
DLE
a. Generalized b. Localized
Differential Diagnosis of SLE:
•
Seborrheic Dermatitis
•
Rosacea
o
Lupus Vulgaris
o
Drug eruption
o
Sarcoidosis
o
Actinic keratosis
o
Others
•
Systemic Lupus Erythematosus
80% Skin involvement ARA (American Rheumatotism Association)
o
Malor Erythema
o
Discoid Erythema
o
Photosensitivity
o
Oral Ulcer(s)
o
 Non Erosive arthritis
o
Serositis (pericorditis or pleaurisy
o
 Nephropathy (Albuminuria or cellular costs)
o
CNS disorders (unexplained seizures or psychosis)
o
Hematologic disorder (Hemolytic anemia with reticulocytosis or leukopenia < 4000/mm3 on two occasions or lymphopenia< 1500/mm3 on two occasions).1
 
o
Immunologic disorders: Any of =+ve/ LE ells preparation or antibodyto native DNA or sm antigen or false +ve serologic test or syphilis(STS).
o
Antinuclear antibody in abnormal titer.
•
Four or more criteria are met serially or simultaneously are needed to have thediagnosis of SLE
•
Cutaneous manifestations :
o
Butterfly erythema
o
Diffuse non scarring alopecia
o
Mucous membranes lesions
o
Cutaneous vasculitis and Telangiectasia
o
Calcinosis Cutis
Systemic manifestation:
•
Arthritis
•
Thrombosis
•
Renal
•
CNS
•
Cardiac
Etiology:
•
Genetic
•
UVL
•
Immune mediated
•
Drugs
o
Hydralazine
o
Procanamide
o
Sulfonamide
o
Isoniazid
o
Anti convulsants
o
Minocycline
Laboratory Findings:
Hemolytic anemia
Thrombocytopenia
Lymphopenia
Leukopenia
ESR 
Coombs’ test may be +ve
Biological false +ve test for syphilis.
IMMUNOLOGIC FINDINGS
1-ANA test 95% of SLE are +ve.>1/3rd of all connective tissue diseases +ve2-LE cell test --> specific but not very sensitive.3-ds-DNA : Anti double stranded DNA ( anti native)DNA – it is specific, but not very sensitive. Indicates high risk of renaldiseases.2
 
4-Antinuclear ribonucleic acid protein (anti-nRNP). Indicate low risk of renaldiseases and good prognosis. Seen in mixed connective tissue disease andSLE.5-Anti-La antibodies +ve and 10-15% of SLE and 30% of Sjogren’s syndrome.6-Antip-Ro antibodies +ve 25% of SLE 40% of Sjogren’s syndrome--> 70% in SCLE--> 75% in Neonatal LE--> 50 - 75% in C2 and C4 deficient LE--> 50% in late onset LE--> 50-60% in Asian patients with LE photosensitivity may striking.7-Serum compliment - low level indicates activity8-Lupus band test --> 70% of lesions of DLE and S/E in normal skin in SLE(twice as common in sun - exposed as in protected skin) +ve protected skin,correlates well with +ve anti ds DNA antibodies and renal disease, andhence with poor prognosis.9-Anti-ssDNA antibody, it is sensitive, but not specific.10-ANA pattern – peripheral is SLE specific11-Anti phospholipid antibodies.Subtypes- Anti-cordiolipin
SCLERODERMA1. Cutaneous Types:
Localized Morphea
Morphea - Lichen sclerosis et atrophicus overlap
Generalized Morphea
Pansclerotic Morphea of Children
Linear Scleroderma.
2. Systemic Types:
Crest SyndromeCalcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly,Telengiectasia
Progressive systemic sclerosis
o
Skin findings
o
Internal involvement
RADIOGRAPHIC FINDINGS
Histology
Differential Diagnosis
Pathogenesis
Treatment
DERMATOMYOSITISSKIN FINDINGS:
Muscle changes
Diagnostic criteria:
1-Symmetrical weakness of limb girdle muscles and anterior flexors of the neck.2-Increased muscle enzymes3
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