CASE PRESENTATION DEPARTMENT OF DVL, KIMS DR.K.RAGHU MOHAN 2ND YEAR PG MD DVL

Transcription

1 CASE PRESENTATION DEPARTMENT OF DVL, KIMS DR.K.RAGHU MOHAN 2ND YEAR PG MD DVL

2 Patient name- XXX Age/Sex - 30yrs/ F Occupation - Home maker Resident - Gundala, Janagom IP NO

3 CHIEF COMPLAINTS Increased pigmentation of face, upper limbs and lower limbs with thickening and dryness of skin since 1 year.

4 HISTORY OF PRESENT ILLNESS Patient was apparently asymptomatic 1 year back then she observed skin tightness initially started on back of right hand which gradually progressed proximally to forearm, face with in 1 to 2 months.

5 She observed multiple pin head sized, pale lesions initially on knuckle area which gradually increased in number seen on both the ears and lower limbs.

6 Then she consulted a local doctor used medication topically and orally for 3 weeks as there was no improvement in the lesions she stopped using them. She noticed swelling of both upper limbs and tightness of fingers since 8 months.

7 Increased pigmentation and thickening of both feet and ankle region from past 3 months associated with on and off swelling History of painful bluish discoloration of fingers on exposure to cold which reverts on rewarming.

8 No history of pruritus, restriction of joints. No h/o difficulty in eating or swallowing, thinning of lips.

9 No h/o morning stiffness or weakness of muscles or impairment at work. No h/o fever, bodyache, myalgia. No h/o any hard raised lesions with chalky discharge / ulcerations.

10 No h/o difficulty in breathing on exertion/at rest/ dry cough /chest pain /palpitations. No h/o reflux / vomiting / bloating /constipation/incontinence/diarrhea/ abdominal pain.

11 No h/o loose stools /passing flatus more frequently. No h/o oliguria/ frothy urine. No h/o headache / visual disturbances / seizures.

12 No h/o difficulty in speaking or taking frequent sips of water, cracking or fissuring at angles of mouth / grittiness in eyes increased at the end of the day /tingling or numbness in hands and feet.

13 No h/o photosensitive rash/oral ulcers. No h/o swelling around the eyes / difficulty in standing from squatting position / combing hair.

14 PAST HISTORY Not a known case of DM /HTN / Epilepsy /Asthma / IHD. Ten years back patient was operated for appendicitis.

15 PERSONAL HISTORY Diet:Mixed Appetite:Decreased Bladder&Bowel:Regular Sleep:Adequate No addictions

16 MENSTRUAL HISTORY Attained menarche at 13 years of age. Hysterectomised 10 years back(? fibroid)

17 OBSTETRIC HISTORY Para 2 live 2 No h/o any complications.

18 FAMILY HISTORY No history of any similar complaints within the family members.

19 DRUG HISTORY Patient used allopathic medication (documents not available) from local doctor one month back. No history of any drug allergies.

20 GENERAL EXAMINATION Patient is conscious, coherent and cooperative,well oriented in time, place and person. Thin built and moderately nourished. PR- 76/min, regular, normal volume BP- 110/80 mm of Hg in supine position RR- 18/min,abdomino-thoracic. Temperature-Afebrile.

21 No Pallor/ Icterus / Clubbing /Cyanosis/ Lymphadenopathy/Pedal Edema.

22 SYSTEMIC EXAMINATION CVS- S1, S2 heard, no murmurs. Respiratory System- BAE +, normal vesicular breath sounds 14 breaths/minute Chest expansion 4cm.

23 P/A soft, no organomegaly. CNS- Higher mental functions- Normal No cranial nerve involvement. No focal neurological deficits

24 CUTANEOUS EXAMINATION Tautness of skin on dorsum of fingers, hands, forearm, arm and feet more on extensor aspect with diffuse hyperpigmentation, induration and xerosis. On face skin is shiny, hyperpigmented and indurated involving both the cheeks, nose and forehead.

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30 Multiple depigmented macules seen on both the knuckles, pinna and extensor aspects of both the legs.

31 Oral aperture 4 finger insertion is normal ORAL AND GENITAL MUCOSA Normal HAIR AND NAILS-Normal

32 PROVISIONAL DIAGNOSIS Tautness of dorsum of fingers, hands and extending proximally. Tautness of feet Reynauds phenomina -positive?diffuse SCLERODERMA, INDURATED STAGE, UNTREATED.

33 DIFFERENTIAL DIAGNOSES GENERALISED MORPHEA EOSINOPHILIC FASCITIS SCLEREDEMA

34 MANAGEMENT AT HOSPITAL INVESTIGATIONS(13/03/2017): CBP:- Hb- 13.1gm% TLC- 8,100/cumm Neutrophils-63% Lymphocytes-30% Eosinophils-04% Monocytes- 2% Basophils -0%

35 Platelet count 2.6lakhs/cumm Smear-normocytic/normochromic ESR 20mm CUE- WNL RBS 80 mg/dl

36 RFT B. urea- 28 mg/dl S. creatinine 0.6 mg/d S uric acid 3.1 mg/dl S Calcium mg/dl S phosphorus- 3.2 mg/dl

37 SERUM ELECTROLYTES S. sodium 141 mmol/l S. potassium - 3.8mmol/L S chloride mmol/l

38 LFT Total bilirubin 0.49 mg/dl Direct bilirubin 0.30 mg/dl SGOT(AST) 31 IU/L SGPT(ALT) 15 IU/L Alkaline phosphatase 144 IU/L Total proteins 6.9 gm/dl Albumin 3.8 gm/dl A/G ratio: 1.23

39 HIV - Non Reactive HBsAg Negative ANTI NUCLEAR ANTI BODY REPORT(ANA): ANA 48 U/ml -POSITIVE

40 USG ABDOMEN No sonological abnormality detected.

41 X RAY PA VIEW OF BOTH HANDS

42 Diffuse osteopenia Evidence of loose bodies noted at metacarpo phalangeal joints Rest of the bones normal

43 PULMONARY FUNCTION TEST- NORMAL Spirometery within normal limits as (FEV1/FVC) % pred > 99 and FVC% pred>80.

44 MDCT SCAN CHEST (PLAIN) No evidence of interstitial fibrosis No evidence of Non specific Interstitial pneumonia.

45 UPPER GI ENDOSCOPY REPORT Esophagus: Lax Lower esophageal sphincter. Stomach:Linear erosions, hyperemia in antrum. Water melon stomach Duodenum: normal Impression: Lax Lower esophageal sphincter. Grade B reflux esophagitis? GAVE(Gastric antral valscular ectasia)

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48 Skin biopsy done on 14/ 03/ *5mm punch biopsy sample was sent from lesional area(right arm) The histopathology report showed the following features: Section studied shows mildly keratotic thinned out epidermis with basal cell layer pigmentation with dermis including papillary dermis shows thickening, hyalinization of collagen fibres with mild perivascular periadnexal focal lymphocytic infiltrate.

49 Blunting of dermal subcutaneous interface is observed. DIGNOSIS:Features compatible with DIFFUSE SCLERODERMA

50 LOW POWER

51 HIGH POWER

52 DIAGNOSIS DIFFUSE SCLERODERMA, INDURATED STAGE WITH WATER MELON STOMACH.

53 TREATMENT (15/03/2017) 1.TAB.METHOTREXATE 5mg test dose given. 2.TAB.RABEPRAZOLE 20mg OD(Before break fast) 3.TADALAFIL 2.5mg OD 4.EMOLLIENT( Petrolatum jelly-for hands and face) 5.TAB.FOLIC ACID 5mg OD(except on day of methotrexate)

54 5.TAB.Calcium carbonate(1250 mg)and vitamin D3 250 IU OD 6.TAB.B- Complex OD

55 FOLLOW UP(After one week) CBP HAEMOGLOBIN 13.3 gm% TLC 7900/cumm NEUTROPHILS 60% PLATELETS 2.43 Lakhs/cu.mm SMEAR-Normocytic/Normochromic

56 LFT TOTAL BILIRUBIN 0.49 mg/dl DIRECT BILIRUBIN 0.30 mg/dl SGOT(AST) 31 IU/L SGPT(ALT) 15 IU/L ALKALINE PHOSPHATASE 144 IU/L

57 1.TAB.METHOTREXATE 7.5 mg (Weekly once) 2.TAB.RABEPRAZOLE 20mg OD Before break fast 3.TAB.FOLIC ACID 5mg OD(except on day of methotrexate)

58 4.TAB.TADALAFIL 2.5mg OD 5.TAB.B COMPLEX OD 6.EMOLLIENT for hands and face 7.TAB. CALCIUM CARBONATE 1250mg+VITAMINE D3 250 IU OD

59 FOLLOW UP(After 10 weeks) CBP Hb gm% TLC- 6,300/cumm Lymphocytes -36% Neutrophils-55% Monocytes 2% Platelet count 2.56lakhs/cumm Smear-normocytic/normochromic

60 LFT TOTAL BILIRUBIN 0.49 mg/dl DIRECT BILIRUBIN 0.30 mg/dl SGOT(AST) 31 IU/L SGPT(ALT) 15 IU/L ALKALINE PHOSPHATASE 144 IU/L

61 FOLLOW UP(After 10 weeks) AT THE TIME OF ADMISSION AFTER 10 WEEKS

62 AT THE TIME OF ADMISSION AFTER 10 WEEKS

63 AT THE TIME OF ADMISSION AFTER 10 WEEKS

64 AT THE TIME OF ADMISSION AFTER 10 WEEKS

65 AT THE TIME OF ADMISSION AFTER 10 WEEKS

66 Patient is on regular follow up till date.

67 THANK YOU