CONNECTIVE TISSUE DISEASES

1. Lupus Erythematosus (LE)

2. Dermatomyositis
3. Scleroderma
4. Rheumatoid
5. Sjogren’s Syndrome
6. Eosinophilic Fasciitis
7. Mixed Connective Tissue Disease (MCTD)
8. Relapsing Polychondritis

LUPUS ERYTHEMATOSUS

I. 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).

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 o Immunologic disorders: Any of =+ve/ LE ells preparation or antibody
to 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 the
diagnosis 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 +ve
2- 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 renal
diseases.

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 4- Antinuclear ribonucleic acid protein (anti-nRNP). Indicate low risk of renal
diseases and good prognosis. Seen in mixed connective tissue disease and
SLE.
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 activity
8- 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, and
hence with poor prognosis.
9- Anti-ssDNA antibody, it is sensitive, but not specific.
10- ANA pattern – peripheral is SLE specific
11- Anti phospholipid antibodies.
Subtypes- Anti-cordiolipin

SCLERODERMA
1. Cutaneous Types:
 Localized Morphea
 Morphea - Lichen sclerosis et atrophicus overlap
 Generalized Morphea
 Pansclerotic Morphea of Children
 Linear Scleroderma.

2. Systemic Types:
 Crest Syndrome
Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly,
Telengiectasia

 Progressive systemic sclerosis

o Skin findings
o Internal involvement

RADIOGRAPHIC FINDINGS
 Histology
 Differential Diagnosis
 Pathogenesis
 Treatment

DERMATOMYOSITIS

SKIN FINDINGS:
Muscle changes

Diagnostic criteria:
1- Symmetrical weakness of limb girdle muscles and anterior flexors of the neck.
2- Increased muscle enzymes
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 a. CPK
b. Transaminase
c. LDH
d. Aldolase
3- Abnormal EMG
4- Characteristic myositis on muscle biopsy
5- The typical dermatologic features:
Two of (1-4) + Dermatologic features make diagnosis probable.
Three + Dermatologic features form of diagnosis.