CLINICAL CASE

VAISHALI

MBBS 2012

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

HISTORY OF PRESENT ILLNESS:Oral lesions

• Multiple erosions and ulcers in the oral cavity and lips whichwere intermittent and recurring.

• Associated with difficulty in chewing and talking.

Skin lesions

• Multiple, red colored lesions over body since 1 yr ; initiallyover the chest which gradually increased in size, thendeveloped 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.

• H/o low grade, continuous fever x 2 days .

• No h/o headache , change in wt/appetite, epigastricpain/nausea/vomiting, chest pain/palpitation/breathlessness,sleep disturbances, altered behavior , B/B dysfunction, bloodtransfusion, drug sensitivity .

PAST HISTORY: k/c/o hypothyroidism ; beingtreated with medication.

• H/o repeated episodes of paralysis since 4 years (hypokalemiawith Renal Tubular Acidosis).

• No h/o TB/Asthma/HTN/any other major chronic illness.

FAMILY HISTORY: No h/o such illness in thefamily.

MENSTRUAL HISTORY: H/o hysterectomy 5

years back.

OCCUPATIONAL HISTORY: used towork in fields.

EXAMINATION• GPE: conscious, co-operative, oriented to TPP, febrile

• PR: 80/min, regular, good volume, vessel wall not palpableBP: 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.

• Lips: multiple erosions were present over both lips ; associated withcrusting. Crust removal caused oozing of blood from the lesions.

• Nails :

Upper Limb Lower Limb

CVS

CNS

Respi. Within normal limits

P/A

Plate Mild yellowish

brown colored

dystrophic nail

plate

NAD

Fold NAD NAD

Cuticle NAD NAD

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

ECG , X-Ray(chest) : Normal

KIDNEY BIOPSY: biopsy specimen sent for

evaluation.

INVESTIGATIONS

Hb 10.4gm%

TLC 7500mm3

DLC 80/15/3/2/0

Plt 1.7 lac/mm3

PBF N/N

U. Protein 1.6gm/day

24-HOUR URINE:

COMPLETE HEMOGRAM:

ESR 112mm/hr

HIV

HBsAg Negative

anti-HCV

SKIN BIOPSY : showed epidermal atrophy

with follicular plugging. The dermis showed

moderate to dense chronic inflammatory

infiltrate.

SEROLOGICAL MARKERS:

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

PROBABLE DIAGNOSIS40 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

CHRONIC CLE

SYSTEMIC LUPUS ERYTHEMATOSUS

SLICC Diagnostic Criteria :

CLINICAL CRITERIA IMMUNOLOGIC

CRITERIA1. 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 manifestatonswithin few weeks

• Localised form: malar rash

• Generalised form: can involvewhole body; systemicmanifestations are present

ACUTE CLE

• Subtypes include: 1.DLE(localised or generalised) 2.Hypertrophic DLE 3.Lupus profundus4.Mucosal LE 5.Chilblain lupus

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 dosecorticosteroids

~If organ threateningSLE, high dosecorticosteroids withimmunosuppressivedrugs.