A 35 years old female

presented with thickening &

tightening of skin, difficulty in
deglutition and non pitting
edema
PRESENTED BY
DR. SHUVO
INTERN DOCTOR
MEDICINE UNIT 1
SOMCH

PARTICULARS OF THE PATIENT

NAME: JOSHNA BEGUM
AGE: 35 YRS
SEX: FEMALE
RELIGION: ISLAM
MARITAL STATUS: MARRIED
OCCUPATION: HOUSEWIFE
ADDRESS: BISHWANATH, SYLHET
DATE OF ADMISSION: 02ND MAY 2015
DATE OF EXAMINATION: 07TH MAY 2015

Chief Complaints
1. Thickening, tightening and
swelling of the skin for 3
months
2. Multiple joint pain for 3
months
3. Burning sensations in chest
& abdomen with difficulty in
swallowing for 3 weeks

History of Present Illness

According to the statement of
the patient, she was reasonably
well 6 months ago. Then she
noticed burning sensations in
3rd and 4th finger of her left
hand. The burning sensations
used to occur intermittently but
she neither noticed any
associated color change in
fingers nor any aggravating
factors. The pain used to relieve

In order to get relief she was admitted

in a private hospital in Sylhet after her
condition was improved after receiving
some medications. But soon she
developed burning sensations in her
right hand fingers.
3 months ago, her both forearms, legs,
wrists, fingers and toes of both hands
and feet started to swell and became
tightened subsequently. Soon she
became unable to make a complete fist
which was associated with pain.

1 month ago, the swelling

appeared in her face and neck
followed by tightening of the
skin which used to restrict her
opening her mouth widely.

She also complained of multiple

joint pain which started appearing
simultaneously involving her right
hand, left wrist, lower back and
both knee joints. The pain was
continuous and had no
relationship with rest or
movement. She did not give any
history of joint swelling, redness,
warmth or deformity and there
was no morning stiffness as well.

For the last 3 weeks, the patient

has been suffering from burning
sensations in her upper abdomen
and chest, difficulty in
swallowing, excessive belching &
early satiety. The difficulty of
swallowing was progressive,
painless, with both solid and
liquid.
Her bowel and bladder habit was
normal. She lost 10 kg wt in last 6

History of Past Illness

Medical
History of MI 5 years ago. She
gave no
history of Hypertension,
Diabetes or CVD.
Surgical
History of cholecystectomy 8
years ago.

Treatment History
She took several drugs for her
recent illness before being
admitted to this hospital but she
could not mention the name of
those drugs.
She gave no history of drug
allergy.

Personal History
She does not chew betel nut.
She is a non
smoker.
Family History
Her father is suffering from
rheumatoid
arthritis. Other members are
healthy.

Socio-economic History
She came from a middle class
family
She used to drink tube well
water
Travel History
She had no significant travel
history

Immunization History
Immunized
Menstrual History
She is a regular menstruating
woman. But
recently she noticed very poor
flow lasting for
1 day only.

Obstetric History
She is having 3 children. All of
them are
healthy.

General Examination

Appearance: Ill looking

Body built: Average
Nutrition: Normal
Decubitus: On choice
Co-operation: Co-operative
Anaemia: Non-anemic
Jaundice: Non-icteric
Cyanosis: Non-cyanosed

Clubbing: Absent
Koilonychia: Absent
Leukonychia: Absent
Dehydration: Absent
Oedema: Present, pitting
Pulse: 80 bpm
BP: 110/80 mmHg
Respiratory rate: 18 b/min
Thyroid gland : Not enlarged
JVP : Not raised
Lymph node: Not palpable

Skin examination
Extremities
Non-pitting edema of fingers,
wrists (Flexor tendon sheaths),
forearms, legs, ankles, feet, face
and neck.
Shiny, thick and tight skin. Distal
skin creases was disappeared. No
erythema or telangiectasia.
There is generalized

There was no thickening of skin

in trunk and above elbows and
knees.
Face and neck
Thinning of lips, furrowing,
microstomia.
Skin of the chest is tight and
thick.

Systemic Examination
Musculoskeletal System
Hands
There was scleroductyly with the
skin of
both hands being smooth, shiny,
tight, thick
and oedematous with
hyperpigmentation.
No telangiectasia was found.

Joints
No swelling, redness, warmth or
deformity was present.
Muscles
No tenderness or wasting was
present.

Abdomen Examination
Inspection
Shape of the abdomen:
Scaphoid
Umbilicus: Centrally placed &
inverted
No engorged veins, visible
pulsation in
epigastrium/peristalsis was
present

Palpation
Soft.
No tenderness, muscle guard
or rigidity & organomegaly, no
tender hepatomegaly, no
shifting dullness or fluid thrill.
Percussion
Tympanic.
Auscultation
Bowel sound was present.

Respiratory Examination
Breath sound is vesicular.
There is no added sound.
Cardiovascular system
examination
No left parasternal heave,
palpable/loud P2

Salient features
Mrs. Josna Begum, 35yrs old, housewife,
married women hailing from Bishwanath,
Sylhet, non-diabetic, non-hypertensive
admitted in this hospital on 2nd may 2015
with the complaints of intermittent
burning sensation in fingers of both
hands for 6 months; gradual thickening,
tightening of skin of both of her forearms,
wrists, hands, legs, ankles, feet as well as
face and neck for 3 months; multiple joint
pain for 3 months and

burning sensation in epigastrium

and chest with difficulty in
deglutition for 3 weeks.
On general examination, there was
non pitting oedema. Her skin was
shiny, thick and tight with distal
skin creases disappeared. There
was no erythema or telangiectasia
in nail fold bed. There is
generalized hyperpigmentation in
the body.

On musculoskeletal system
examination,
there was scleroductyly with the
skin of both
hands being smooth, shiny, tight,
thick and
oedematous with
hyperpigmentation. No
telangiectasia was found. There
was no swelling, redness or
warmth in her joints. No muscle
wasting were present.

Provisional diagnosis

Limited Cutaneous
Systemic Sclerosis
(LCSS)

Differential
diagnosis

Investigations
Complete blood count
Hb% : 14 g/dl
ESR: 29 mm in 1st hr
WBC : 11800/cmm
Neutrophil : 59%
Lymphocyte: 35%
Monocyte : 03%
Eosinophil : 03%

Basophil : 0%
Platelet : 155000/cmm

CRP: 10.1 mg/L [<10 mg/L]

CPK: 95 U/L []
RA: Negative (8.6 IU/ml) [<20
IU/ml]
Elisa for ANA: 160.7 IU/ml [Cut off
rate 1.50, ]
C-ANCA: <1 U/ml [Normal <12,
Equivocal 12-18, Positive >18]
P-ANCA: <1 U/ml [Normal <12,
Equivocal 12-18, Positive >18]

Skin biopsy (06/05/2015)

Section of skin show deposition
of thick collagen bundle in
dermis. Skin adnexae are
reduced. Mild perivascular
infiltrate of chronic
inflammatory cells are present.
Epidermis reveals mild
hyperkeratosis.
Comment: Compatible with

Urine R/E
Pus cell: 4-5/HPF
Epithelial cells: 2-3/HPF
RBC, protein, suger: Nil
pH: Acidic
ECG: Suggestive of an old
anterior MI
RBS: 5 mmol/L
Serum Creatinine: 0.75mg/dl

Total Lipid Profile

Total cholesterol: 162 mg/dl
Triglyceride: 270 mg/dl
HDL : 41 mg/dl
LDL : 67 mg/dl

Hormone Analysis
Serum T3: 1.50 ng/ml
Serum T4: 8.26 mcg/dl
Serum TSH: 1.94 mcIU/ml

Clinical Diagnosis

Thank you