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Crimean State Medical University named after

S.I Georgievsky
DEPARTMENT OF
INTERNAL MEDICINE N.2

Head Of Department : PROF. BELAGLAZOV LAMIDIR


Teacher

: MADAM MAKSIMOVA ELENA VLADIMIZOVNA

CASE REPORT
Name of patient : Kadirova Zuloiha Yakubovna
Age
: 57 years old
Clinical Diagnosis
Main disease: Scleroderma, acute, 1st degree activity, diffuse form with
skin rashes, Raynauds syndrome and telangiectasia
Complication: Accompanying disease: -

Students Name : TAN WAN SHIN


Group : 629
Date of examination : 22-10-2009

Passport data
Name: Kadirova Zuloiha Yakubovna
Age: 57
Year of birth: 15/3/1952
Address: Okvaska Village, Kucatava Street, Simferopol.
Professional: Not working
Nationality: Ukrainian

Complaints
Patient complains of skin rashes on chest, abdomen, thighs and inguinal region. She also complains of
paraesthesia of fingers of both hands. Moreover, sometimes when her hands exposed to cold, she felt
periodic pain on fingers and she found that her fingers first became bluish purplish, and then they turn
pale and finally became red. She also complains that recently she cant sleep well at night.

Anamnesis morbi
Patient realized the present of rashes since July 2009. But, at that time, the rashes only located on the
chest. Later, she found that the same type of rashes started to appear on abdomen, later on thighs and
inguinal regions. Others regions and extremities are spared. These rashes didnt cause any pain and
itchiness. So, she just ignored it until recently she started to worry and finally came to Semashka
Hospital. She denied of taking any drugs for the rashes before this.

Anamnesis vitae
According to the patient, none of her relatives and parents suffered this before. She dont smoke and
drink. She has no allergy to any food or drug. Her family also doesnt have any allergy. She didnt have
any childhood diseases and chronic diseases like hypertension and diabetes mellitus. She also denied of
any infectious diseases. She didnt encounter any trauma before. She also didnt have any blood
transfusion. She never underwent any operations.

General examination
Generally, the patients status is satisfactory. While interviewing, her posture was in normal position.
Patient is conscious while interview. She corresponds to the questions asked. Her appearance
corresponds to her age. Her body structure is normal. Her body constituition is normosthenic.
Temperature of body : 36.7C (normal)
Blood pressure
: 120/70 mmHg
Pulse
: 82 beats/min (regular)
Respiratory rate
:19/min

System review
Skin
During inspection, the skin of the patient is normal pinkish colour but there are lots of rashes located on
chest, abdomen, thighs and inguinal regions. On extremities and other regions, these rashes are absent.
The rashes are pinkish, patchy and tends to confluent. They became pale when pressure was applied.
The patients skin humidity is normal with normal turgor and elasticity. There is no post-operative scar.
Subepidermal adipose tissues accumulate moderately in the abdomen. Edema is not present on both legs.
No visible varicoses of vein on the calfs.

Patients hands are not very warm. There are slight edema on both hands until metacarpophalangeal
joint. Patient can flex and extend all her fingers. Her fingers appeared to be normal without any
deformation and without any colour changes. Nails are normal without any brittleness or pitting.
Patients hair corresponds to her age, not brittle and not dry.
During inspection of face, there are a few visible dilated vessels near the nose bridge, under both eyes.
Mucous membrane of conjunctiva, nasal and oral cavity appears to be pale - pink.
Fatty tissue
The skin fold thickness under the scapula is approximately 1.5 cm.
Lymph nodes
Left axillary lymph node is enlarged, around 1.5 cm. Submandibular lymph nodes are determined with
size from 0.5-1.0 cm in diameter. These lymph nodes, including the enlarged left axillary lymoh node
are rounded form with smooth surface, elastic consistence, and mobile, not fix to skin. Cervical,
supraclavicular and subclavicular lymph nodes are impalpable on both sides. Others lymph nodes are
impalpable.
Locomotor system
All active and passive movements of the joints are full. No pathologic deformation is seen in the spinal
column. Its function is full.
The muscles are developed moderately, tone and muscle force are identical on both sides. Palpation and
tapping of the bones are painless. Joints are regular in shape, painless during palpation and movements.
The muscles, bone and joints are in a generally good condition.
Respiratory system
Patient doest complaint of dyspnoe during physical exertion or at rest. Cough and pain is absent in the
patient. During inspection, patient breathes through her nose. Her chest shape is normal without
pathological signs. On her chest, there are patches of skin rash, they are pinkish in coloured, and became
pale on pressure.Epigastria angle 90. Chest is symmetrical at supra and subclavicular space. Direction
of ribs is normal. The patient has a normostenic and regular chest. Respiratory rate is 19 per min and it is
regular. Movement of the chest is symmetrical during respiration.
During palpation, there is no tenderness noted along the ribs, intercostal spaces, trapezoid muscles and
intercostal nerves points. Resistance of chest at longitudinal and transverse direction is normal. Vocal
fremitus at the anterior, lateral and posterior is symmetrical and normal.
At comparative percussion, clear lung sounds are heard over symmetrical points.
Over auscultation, vesicular breathing is heard with longer inspiration and shorter expiration. No
adventitia sounds were heard.
Cardiovascular system
Pulse rate :
82 beats per min regular
Blood pressure :
120/70 mm Hg
During inspection, no visible pulsation is noted at the carotid, jugular or temporal vessels. There is no
protrusion in the heart area (cardiac humpback), no pulsation is visible apex beat or at any other chest
zones. No epigastric pulsation is noted. No cyanosis was seen. No edema is noted during inspection .
During palpation, apex beat is 1cm medially from left midclavicular line along 5 th intercostals space.
Cats murmur phenomenon is not determined.
On auscultation of the heart, 2 sounds are heard at all points. The sounds are rhythmic, sounds are heard
clear, murmurs are absent.

Digestive System
The patient does not have any difficulty in swallowing or passing food through the esophagus. No
hypersalivation.
The mouth is in a generally clean condition, without any unpleasant smells. The size of the patients
tongue is usual. The tongue is moist and clean. The tongue is in a generally normal state. Her teeth and
gums are healthy. Mucous membrane of the mouth is light pink colour.
The shape of the abdomen is normal and symmetrical. Tympanic sound is heard over all parts of bowel
in the abdomen. Dull sound can be heard over the part where located liver and spleen.
Skin of the abdomen is soft and painless. There are patches of pink coloured, blanchable skin rashes on
the abdomen. Hernias and superficial tumours are not palpable. Sklyarovs, Mendels and ShchetkinBlumbergs symptoms are negative.
The sigmoid colon can be palpated in the left iliac region. It is cylindrical in form, soft and painless
during deep palpation.
By using deep palpation, the caecum can be palpated in the right iliac region. The caecum is palpated as
a smooth, soft cylinder and there is no pain. Greater curvature of the stomach can be palpated. The
stomach is soft during palpation and painless.
Liver is normal during palpation. There are no sign of enlargement of the liver, The size of the liver
according to Kurlov and by Obraztsov is normal. Gall bladder is not palpable. Symptoms of pressing
and tapping of gall bladder are negative and all tenderness points of gall bladder are negative. (Keras
symptom, Murphys symptom, Ortners symptom, Lepenes symptom and Vasilenkos symptom are
negative.)
Pancreas is not palpable. Kachas point and choledochopancreatic point are negative.
During palpation the abdomen is soft, painless. Symptoms of irritation of peritoneum (shetkin
Blumberg)is negative. During auscultation of the abdomen, rumbling sound is heard.
Urinary System
There is no pain in the groins, sacrum and the supra-pubic area. The patient has normal urination. The
amount of urine is normal. There is no pain during urination. Colour of the urine is normal, light yellow
color. Edema, hyperemia and swelling of the groins are absent. The kidneys are not palpable. When
checked, Pasternatsky symptom is negative for both sides of the lumbar region. The urinary bladder is
not palpable.
Endocrine system
During inspection, changes of the eyes (exophthalmus or enophthalmus) are not visible. Ocular
symptoms like Grafes, Kochers, Moebius, Stelwags are negative. During palpation, the thyroid gland
is not enlarged. Its isthmus is palpated as soft, painless and 1cm in diameter.
Blood system
The patient doesnt complains of general weakness, dizziness, fatigue, palpitation. The patient does not
have any perversion of taste or olfaction. Haemorrhagic spots on the skin and mucosa are not
present.The spleen is not palpable.
Nervous system and psychic state
The consciousness of the patient is clear. The patients manner of speech is clear and correct. The patient
is able to think in a logical and rational manner.

There are no muscular cramps on the extremities of the patient. The patient does not have any numb
sensations on his leg and hands. She had good coordination and normal gait.
Any forms of paralyses and paresis is absent in the patient. The condition of the patients pupils is
normal. The pupils react to light.

Status localis
During inspection, the skin of the patient is normal pinkish colour but there are lots of rashes located on
chest, abdomen, thighs and inguinal regions. On extremities and other regions, these rashes are absent.
The rashes are pinkish in coloured, patchy and tends to confluent. They became pale when pressure was
applied.
Patients hands are not very warm. There are mild edema on both hands until metacarpophalangeal joint.
Patient can flex and extend all her fingers. Her fingers appeared to be normal without any deformation
and without any colour changes. Nails are normal without any brittleness or pitting.
During inspection of face, there are visible dilated vessels near the nose bridge, under both eyes.

Plan of investigations
Nonspecific :
1. General blood analysis
2. Urinalysis
3. Biochemical test
4. Chest X-ray
5. ECG and Echocardiography of heart.
6. Ultrasound of thyroid gland, serum TSH and T3,T4 level
Specific(Diagnostic):
1. Anti Scl-70 ( Anti Topoisomerase ) antibody
Results
Complete blood Count
Erythrocyte
4.2x1012/L
Hemoglobin
141g/L
Color Index
0.9
Leucocytes
6x109/L
Eosinophils
5%
Stab
5%
Segmented
59%
Lymphocyte
29%
Monocytes
2%
ESR
5mm/hr
Conclusion: Normal

Biochemical test of blood


Protein
70g/l
Bilirubin
8.29 mmol/L
ALAT
0.84 mmol/L
ASAT
0.25 mmol/L
Tymol Test 1.9
Conclusion: Normal

Urine analysis
Color
Specific gravity
Transparency
Reaction
Protein
Glucose
Leukocytes
Epithelium
Conclusion : Normal

Yellow,
transparent
1019
Clear
Acidic
1-2
0-1

Chest X-ray : To confirm absent of fibrosis of lung.


ECG : ST depression in V6 May be a sign of myocardial sclerosis, so further investigation by
Echocardiography is highly recommened.
Ultrasound of thyroid gland, serum TSH and T3,T4 level : To exclude hypothyroidism.

Conclusion
The patient started to develop skin rashes which are pinkish, patchy and blanchable since July 2009.
This shows an acute onset of disease. These skin rashes first located on chest, later started to involve
abdomen, thighs, and inguinal region. This suggests diffuse form of disease.
Raynauds syndrome also appeared in this patient based on her complaint of periodic fingers ache and
changing of colour from cyanosis to pale to red when exposed to cold. There are mild edema on both
hands until metacarpophalangeal joint. This indicate limited form because edema only involve hands.
Palmar erythema present on both palms. During inspection of face, there are sign of telangiectasia
few visible dilated vessels near the nose bridge, under both eyes.
Most probably, scleroderma is my diagnosis. Due to 2 positive criteria from CREST, which are
Raynauds syndrome and telangiectasia. All symptoms and signs are mildly express only, so the disease
might just in the initial stage 1st degree activity.

Substantiation of diagnosis
Main disease: Scleroderma, chronic 1st degree activity, limited form with
Raynauds syndrome and telangiectasia
Complication: Accompanying disease: -

Differential Diagnosis:
Hypothyroidism :

Immunopathogenesis of Scleroderma:
Characterized by 3 phases :
1. Vascular : First, endothelial cells of small vessels ( e.g: small arteries, arterioles, capillaries) are
damage. This lead to release of various cytokines (e.g: endothelin-1 leads to vasoconstriction). This
continous intima damage will increase vascular permeability, leading to cellular activation and
migration of cells into extracellular matrix which then release mediators like IL1,4,6,8 , TGF-b.
These will activate the fibroblasts, lead to fibroblast proliferation.
Vessels damage will produce widespread obliterative arterial lesions and future ischemia.
2. Fibrotic : Fibroblasts increase synthesis of Collagen type 1 , III , fibronectin and
glycosaminoglycans. This lead to fibrosis in lower dermis of skin and internal organ.
3. Humoral Immunity : Autoimmune antibody (e.g : ANA ) start to act on extracellular matrix.

Treatment
Regime: Free, avoid cold.
Diet: No. 15 (physiological diet) intake of food with high vitamin A,C,E and mineral salt, high caloric
food is recommend, 3 meals per day, timely intake of food.

1) Calcium Channel Blocker for Raynauds phenomenon


Rp. : Tab. Corinfar 0.01 N30
D.S. tab. PO 1t/day.
Indication :
Contraindication :
2) Anti fibrotic agent
Rp.: Tab. Tamoxifeni 0.02
D.t.d. No. 30
S. Taken 2 t/d PO after meal.
Mechanism of action :
Indication
Contraindications

:
: Pregnancy.

Complications

3) Electrophoresis with Lydase (Physiotherapy )

Prognosis

In aspect of life, prognosis is favorable, she can carry on her normal daily activity without any
problem as long as fibrosis activity is undercontrol and no complication ( no internal organs involved)
In aspect of health, it is unfavorable, because scleroderma is a chronic disease and treatments are only
symptomatic.
In aspect of her work, it is unfavorable, because in chronic disease, patient easily feel tired. Thus she
need to take more rest.