CASE PRESENTATIONDEPARTMENT OF DVL,KIMS

DR.K.RAGHU MOHAN2ND YEAR PG

MD DVL

Patient name- XXXAge/Sex - 30yrs/ FOccupation - Home makerResident - Gundala, JanagomIP NO-201634187

CHIEF COMPLAINTS

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

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.

• 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.

•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.

• 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.

• No history of pruritus,restriction of joints.

•No h/o difficulty in eating or swallowing, thinning of lips.

•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.

• 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.

•No h/o loose stools /passing flatus more frequently.

•No h/o oliguria/ frothy urine.

•No h/o headache / visual disturbances / seizures.

• No h/o difficulty in speaking or takingfrequent 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.

• No h/o photosensitive rash/oral ulcers.

• No h/o swelling around the eyes / difficulty in standing from squatting position / combing hair.

PAST HISTORY• Not a known case of DM /HTN / Epilepsy

/Asthma / IHD.

• Ten years back patient was operated forappendicitis.

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

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

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

FAMILY HISTORY• No history of any similar complaints

within the family members.

DRUG HISTORY• Patient used allopathic medication

(documents not available) from local doctor one month back.

• No history of any drug allergies .

GENERAL EXAMINATION• Patient is conscious, coherent and co-operative,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.

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

SYSTEMIC EXAMINATIONCVS- S1, S2 heard, no murmurs.Respiratory System-BAE +, normal vesicular breath sounds14 breaths/minuteChest expansion 4cm

.

P/A – soft, no organomegaly.CNS- Higher mental functions- Normal

No cranial nerve involvement.No focal neurological deficits

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 andindurated involving both the cheeks, nose andforehead.

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

• Oral aperture 4 finger insertion is normal

• ORAL AND GENITAL MUCOSA –Normal

• HAIR AND NAILS-Normal

PROVISIONAL DIAGNOSISTautness of dorsum of fingers, hands and

extending proximally.Tautness of feetReynauds phenomina -positive ?DIFFUSE SCLERODERMA,

INDURATED STAGE,UNTREATED.

DIFFERENTIAL DIAGNOSES• GENERALISED MORPHEA• EOSINOPHILIC FASCITIS• SCLEREDEMA

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

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

• RFTB. urea- 28 mg/dlS. creatinine – 0.6 mg/dS uric acid – 3.1 mg/dlS Calcium- 10.5 mg/dlS phosphorus- 3.2 mg/dl

SERUM ELECTROLYTESS. sodium – 141 mmol/LS. potassium - 3.8mmol/LS chloride - 101 mmol/L

LFTTotal bilirubin 0.49 mg/dlDirect bilirubin 0.30 mg/dlSGOT(AST) – 31 IU/LSGPT(ALT) – 15 IU/LAlkaline phosphatase 144 IU/L• Total proteins – 6.9 gm/dlAlbumin 3.8 gm/dlA/G ratio: 1.23

• HIV - Non Reactive• HBsAg – Negative•ANTI NUCLEAR ANTI BODY REPORT(ANA):

ANA 48 U/ml -POSITIVE

USG ABDOMENNo sonological abnormality detected.

X RAY PA VIEW OF BOTH HANDS

Diffuse osteopeniaEvidence of loose bodies noted at meta-carpo phalangeal jointsRest of the bones normal

PULMONARY FUNCTION TEST- NORMAL

• Spirometery within normal limits as (FEV1/FVC)% pred > 99 andFVC% pred>80.

MDCT SCAN –CHEST (PLAIN)No evidence of interstitial fibrosisNo evidence of Non specific Interstitial pneumonia.

UPPER GI ENDOSCOPY REPORTEsophagus: 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)

Skin biopsy done on 14/ 03/ 2017.• 5*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 perivascularperiadnexal focal lymphocytic infiltrate.

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

LOW POWER

HIGH POWER

DIAGNOSIS• DIFFUSE SCLERODERMA,

INDURATED STAGE WITH WATER MELON STOMACH.

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)

• 5.TAB.Calcium carbonate(1250 mg)andvitamin D3 250 IU OD

• 6.TAB.B- Complex OD

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

•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

1.TAB.METHOTREXATE 7.5 mg (Weekly once)2.TAB.RABEPRAZOLE 20mg ODBefore break fast3.TAB.FOLIC ACID 5mg OD(except on day of methotrexate)

4.TAB.TADALAFIL 2.5mg OD5.TAB.B COMPLEX OD6.EMOLLIENT for hands and face7.TAB. CALCIUM CARBONATE

1250mg+VITAMINE D3 250 IU OD

FOLLOW UP(After 10 weeks)CBPHb- 12.5 gm%TLC- 6,300/cummLymphocytes -36%Neutrophils-55%Monocytes 2% Platelet count – 2.56lakhs/cummSmear-normocytic/normochromic

•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

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

AT THE TIME OF ADMISSION AFTER 10 WEEKS

AT THE TIME OF ADMISSION AFTER 10 WEEKS

AT THE TIME OF ADMISSION AFTER 10 WEEKS

AT THE TIME OF ADMISSION AFTER 10 WEEKS

• Patient is on regularfollow up till date.

THANK YOU