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CASE PRESENTATIONDEPARTMENT OF DVL,KIMS
DR.K.RAGHU MOHAN2ND YEAR PG
MD DVL
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Patient name- XXXAge/Sex - 30yrs/ FOccupation - Home makerResident - Gundala, JanagomIP NO-201634187
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CHIEF COMPLAINTS
• Increased pigmentation of face, upper limbs and lower limbs with thickening and dryness of skin since 1 year.
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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.
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• 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.
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•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.
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• 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.
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• No history of pruritus,restriction of joints.
•No h/o difficulty in eating or swallowing, thinning of lips.
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•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.
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• 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.
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•No h/o loose stools /passing flatus more frequently.
•No h/o oliguria/ frothy urine.
•No h/o headache / visual disturbances / seizures.
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• 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.
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• No h/o photosensitive rash/oral ulcers.
• No h/o swelling around the eyes / difficulty in standing from squatting position / combing hair.
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PAST HISTORY• Not a known case of DM /HTN / Epilepsy
/Asthma / IHD.
• Ten years back patient was operated forappendicitis.
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PERSONAL HISTORY• Diet:Mixed• Appetite:Decreased• Bladder&Bowel:Regular• Sleep:Adequate• No addictions
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MENSTRUAL HISTORY• Attained menarche at 13 years of age.• Hysterectomised 10 years back(? fibroid)
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OBSTETRIC HISTORY• Para 2 live 2• No h/o any complications.
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FAMILY HISTORY• No history of any similar complaints
within the family members.
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DRUG HISTORY• Patient used allopathic medication
(documents not available) from local doctor one month back.
• No history of any drug allergies .
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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.
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• No Pallor/ Icterus / Clubbing /Cyanosis/Lymphadenopathy/Pedal Edema.
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SYSTEMIC EXAMINATIONCVS- S1, S2 heard, no murmurs.Respiratory System-BAE +, normal vesicular breath sounds14 breaths/minuteChest expansion 4cm
.
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P/A – soft, no organomegaly.CNS- Higher mental functions- Normal
No cranial nerve involvement.No focal neurological deficits
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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.
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• Multiple depigmented macules seen on both the knuckles, pinna and extensor aspects of both the legs.
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• Oral aperture 4 finger insertion is normal
• ORAL AND GENITAL MUCOSA –Normal
• HAIR AND NAILS-Normal
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PROVISIONAL DIAGNOSISTautness of dorsum of fingers, hands and
extending proximally.Tautness of feetReynauds phenomina -positive ?DIFFUSE SCLERODERMA,
INDURATED STAGE,UNTREATED.
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DIFFERENTIAL DIAGNOSES• GENERALISED MORPHEA• EOSINOPHILIC FASCITIS• SCLEREDEMA
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MANAGEMENT AT HOSPITAL•INVESTIGATIONS(13/03/2017):CBP:-Hb- 13.1gm%TLC- 8,100/cummNeutrophils-63%Lymphocytes-30%Eosinophils-04%Monocytes- 2%Basophils -0%
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• Platelet count – 2.6lakhs/cumm• Smear-normocytic/normochromic•ESR – 20mm• CUE- WNL• RBS – 80 mg/dl
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• 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
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SERUM ELECTROLYTESS. sodium – 141 mmol/LS. potassium - 3.8mmol/LS chloride - 101 mmol/L
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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
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• HIV - Non Reactive• HBsAg – Negative•ANTI NUCLEAR ANTI BODY REPORT(ANA):
ANA 48 U/ml -POSITIVE
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USG ABDOMENNo sonological abnormality detected.
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X RAY PA VIEW OF BOTH HANDS
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Diffuse osteopeniaEvidence of loose bodies noted at meta-carpo phalangeal jointsRest of the bones normal
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PULMONARY FUNCTION TEST- NORMAL
• Spirometery within normal limits as (FEV1/FVC)% pred > 99 andFVC% pred>80.
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MDCT SCAN –CHEST (PLAIN)No evidence of interstitial fibrosisNo evidence of Non specific Interstitial pneumonia.
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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)
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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.
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Blunting of dermal subcutaneous interface is observed.DIGNOSIS:Features compatible with “DIFFUSE SCLERODERMA”
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LOW POWER
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HIGH POWER
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DIAGNOSIS• DIFFUSE SCLERODERMA,
INDURATED STAGE WITH WATER MELON STOMACH.
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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)
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• 5.TAB.Calcium carbonate(1250 mg)andvitamin D3 250 IU OD
• 6.TAB.B- Complex OD
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FOLLOW UP(After one week)•CBP•HAEMOGLOBIN 13.3 gm%•TLC 7900/cumm•NEUTROPHILS 60%•PLATELETS 2.43 Lakhs/cu.mm•SMEAR-Normocytic/Normochromic
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•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
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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)
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4.TAB.TADALAFIL 2.5mg OD5.TAB.B COMPLEX OD6.EMOLLIENT for hands and face7.TAB. CALCIUM CARBONATE
1250mg+VITAMINE D3 250 IU OD
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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
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•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
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FOLLOW UP(After 10 weeks)AT THE TIME OF ADMISSION AFTER 10 WEEKS
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AT THE TIME OF ADMISSION AFTER 10 WEEKS
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AT THE TIME OF ADMISSION AFTER 10 WEEKS
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AT THE TIME OF ADMISSION AFTER 10 WEEKS
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AT THE TIME OF ADMISSION AFTER 10 WEEKS
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• Patient is on regularfollow up till date.
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THANK YOU