A 40-year-old female presented with recurrent oral erosions for 7 years and raised red skin lesions over her body for 1 year. Examination found discoid skin plaques and oral ulcers. Laboratory tests showed positive ANA, low complement levels, and proteinuria. A skin biopsy demonstrated findings consistent with a connective tissue disease. She was diagnosed with systemic lupus erythematosus based on her skin and oral lesions, photosensitivity, positive serologies, and proteinuria. Her symptoms of renal tubular acidosis and hypokalemic paralysis also indicate an overlap with secondary Sjogren's syndrome.

1. CLINICAL CASE
VAISHALI
MBBS 2012

2. A 40 year old female patient
presented with the complaints of:
1. Multiple recurrent erosions on
lips & buccal mucosa x 7 years
2. Multiple, raised red colored
lesions over chest, back, both
Upper Limbs & legs x 12 months
3. Aggravation of the symptoms
x 10 days

3.  HISTORY OF PRESENT ILLNESS:
Oral lesions
• Multiple erosions and ulcers in the oral cavity and lips which
were intermittent and recurring.
• Associated with difficulty in chewing and talking.
Skin lesions
• Multiple, red colored lesions over body since 1 yr ; initially
over the chest which gradually increased in size, then
developed over B/L upper limbs & legs since 3 months;
associated with itching.
• H/o photosensitivity (lesions became red and painful on
sun exposure).
• H/o increased hair loss , joint pain in shoulders,
knee joints; not associated with swelling.

4. • H/o low grade, continuous fever x 2 days .
• No h/o headache , change in wt/appetite, epigastric
pain/nausea/vomiting, chest pain/palpitation/breathlessness,
sleep disturbances, altered behavior , B/B dysfunction, blood
transfusion, drug sensitivity .
PAST HISTORY: k/c/o hypothyroidism ; being
treated with medication.
• H/o repeated episodes of paralysis since 4 years (hypokalemia
with Renal Tubular Acidosis).
• No h/o TB/Asthma/HTN/any other major chronic illness.
FAMILY HISTORY: No h/o such illness in the
family.
MENSTRUAL HISTORY: H/o hysterectomy 5
years back.
OCCUPATIONAL HISTORY: used to
work in fields.

5. EXAMINATION
• GPE: conscious, co-operative, oriented to TPP, febrile
• PR: 80/min, regular, good volume, vessel wall not palpable
BP: 124/80 mmHg RR: 18/min
• O/E: multiple, erythematous, P- I- Cy- Cl- JVPNR LAP- PE-discoid
plaques with adherent scales
and well-defined hyperpigmented
border present over chest , lower
back, B/L upper limbs & legs.Carpet
Tack sign was positive. Grattage test
and Auspitz sign were negative.
• Oral mucosa: multiple ulcerations
and superficial erosions were present
on buccal mucosa and palate.

6. • Lips: multiple erosions were present over both lips ; associated with
crusting. Crust removal caused oozing of blood from the lesions.
• Nails :
Upper Limb Lower Limb
Plate Mild yellowish
brown colored
dystrophic nail
plate
NAD
Fold NAD NAD
Cuticle NAD NAD
CVS
CNS
Respi. Within normal limits
P/A

7. DDX POSITIVE FINDINGS
DISSEMINATED DLE •Annular discoid lesions present
over chest , trunk , extremities
•Positive Carpet Tack sign
•Oral lesions
•Photosensitivity
SLE •Alopecia
•Discoid rash
•Oral ulcers
•Photosensitivity
PSORIASIS •Discoid plaques with scales
SUBACUTE CUTANEOUS
LUPUS ERYTHEMATOSUS
•Skin lesions
•Photosensitivity
•Non-scarring alopecia

8. INVESTIGATIONS
KIDNEY BIOPSY: biopsy specimen sent for
evaluation.
ECG , X-Ray(chest) : Normal
COMPLETE HEMOGRAM:
Hb 10.4gm%
TLC 7500mm3
DLC 80/15/3/2/0
Plt 1.7 lac/mm3
PBF N/N
24-HOUR URINE:
U. Protein 1.6gm/day
HIV
HBsAg Negative
anti-HCV
ESR 112mm/hr
SKIN BIOPSY : showed epidermal atrophy
with follicular plugging. The dermis showed
moderate to dense chronic inflammatory
infiltrate.
SEROLOGICALMARKERS:
1.ANA by IF: +ve(1:640 titre); speckled pattern
2.Antibodies to dsDNA (patient refused
3.Complement (C3,C4)levels for the tests)
PBF for LE cell : Negative
RFT
LFT Normal
S. Electrolytes
RBS

9. PROBABLE DIAGNOSIS
40 year female patient with following findings:
 Skin lesions characteristic of DLE (Discoid Rash)
 Oral ulcers
 Photosensitivity
 Positive ANA titre
 Proteinuria (>0.5 g/day)
--is a case of Systemic Lupus Erythematosus as per the
SLICC Criteria.
 RTA with hypokalemic paralysis and speckled IF pattern of
ANA indicate:
? Overlap syndrome of SLE with secondary
Sjogren’s Syndrome

10. SYSTEMIC LUPUS ERYTHEMATOSUS
CHRONIC
CLE
SLICC Diagnostic Criteria :
CLINICAL CRITERIA IMMUNOLOGIC
CRITERIA
1. Acute cutaneous lupus
2. Chronic cutaneous
lupus
3. Oral or nasal ulcers
4. Non-scarring alopecia
5. Arthritis
6. Serositis
7. Renal dysfunction
8. Neurologic
dysfunction
9. Hemolytic anaemia
10.Leukopenia
11.Thrombocytopenia
(<100,000/mm3)
1. ANA
2. Anti-DNA
3. Anti-Sm
4. Antiphospholipid Ab
5. Low Complement (C3,
C4, CH50)
6. Direct Coombs’ test
• Occurs after sun exposure;
followed by systemic manifestatons
within few weeks
• Localised form: malar rash
• Generalised form: can involve
whole body; systemic
manifestations are present
ACUTE
CLE
• Subtypes include:
1.DLE(localised or generalised)
2.Hypertrophic DLE
3.Lupus profundus
4.Mucosal LE
5.Chilblain lupus

11. TREATMENT
CUTANEOUS
MANIFESTATIONS
~Avoid sun exposure
and use sunscreens
with high SPF.
~Cosmetic camouflage
can be used.
~Balanced diet low in
fat.
~ Topical steroids
~ Intralesional steroids
~Antimalarials
(hydroxychloroquine)
~ Oral steroids
SYSTEMIC
MANIFESTATIONS
~Conservative treatment
+ low dose
corticosteroids
~If organ threatening
SLE, high dose
corticosteroids with
immunosuppressive
drugs.