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PEMPHIGUS VULGARIS
A CASE PRESENTATION BY,
ZAHED ULLA KHAN,
Pharm.D Intern,
H.S.K College of Pharmacy
SNMC & H.S.K Hospital and research centre,
Bagalkote.
AUTOIMMUNE
VESCICULOBULLOUS
DERMATOLOGICAL MANIFESTATION AFFECTIING EPIDERMIS
CHARECTERIZED BY ACANTHOLYSIS
INTRODUCTION
AUTOIMMUNE
ACCOUNTS FOR 70% OF PEMPHIGUS SPECTRUM
DUE TO IGG ANTIBODIES
ALL GENDERS EQUALLY AFFECTED
MEAN AGE IS 30-45 Years
RARELY SEEN IN CHILDERN
RARELY DRUG INDUCED (MAY BE DUE TO PENICILLIN, ACE INHIBITORS)
JEWS, INDIANS,MEDITERANIAN POPULATION
INCIDENCE RATE: 0.5-3.3/Yr, PER 100,000 POPULATION (1)
AETIOLOGY AND EPIDEMIOLOGY
GENETIC PREDISPOSITION
HERPES INFECTION
UNDERLAYING AUTO IMMUNE DISORDER
DRUG SENSITIVITY (RARE)
RADIATION
RISK FACTORS
PATHOPHYSIOLOGY
RUPTURE OF JUNCTIONS
RELEASE OF CELLULAR FLUID
FORMATION OF BULLA
RUPTURE LEADING TO EROSION
SEPSIS
CUTANEOUS EXAMINATION ( NIKOLOSKY’S AND INDIRECT NIKOLOSKY’S)
BIOPSY TZANK SMEAR IMMUNOFLUROSCENCE
DIAGNOSIS
Demiographic Data
Name: Mrs. RSG Age: 36
Sex :F
Dept. SKIN Unit : A
DOA: 6/01/2022 DOD: 27/01/2022
CASE PRESENTATION:
C/O FLUID FILLED LESION SINCE 1 YEAR, INSOIDIOUS AND PROGRESSIVE
REASON FOR ADMISSION
NOT K/C/O HTn, DM, TB, EPILEPSY
PAST MEDICAL HISTORY
THE PATIENT WAS APPARENTLY ALRIGHT ONE YEAR AGO, THEN DEVELOPED FLUID FILLED LESION OVER TRUNK,AND
ON UPPER EXTRMITIES WHICH WERE SPREADING
THE BULLAE LATER RUPTURED TO FORM EROSIONS ASSOCIATED WITH A FEW CRUSTED LESIONS OVER BODY
EROSION + OVER MOUTH AND PALATE
NO Hx OF IBR, DRUG INTAKE, PHOTOSENSITIVITY, JOINT PAIN, WEIGHTLOSS, RASH.
HISTORY OF PRESENT ILLNESS
NAD
SOCIO FAMILIAL HISTORY
Moderately built and nourished
Conscious and oriented
PICCLE : -ve
B.P – 120/80 mmhg
P.R - 80 bpm
GENERAL PHYSICAL EXAMINATION
CVS :
RS:
NAD
CNS :
P/A:
SYSTEMIC EXAMINATION
MULTIPLE EROSIONS +OVER TRUNK, EXTREMITIES, BACK, FACE, AND A FEW LESIONS WERE WITH THICK
CRUST.
MULTIPLE EROSION + ORAL MUCOSA
NAIL, HAIR, GENITAL MUCOSA : NORMAL.
CUTANEOUS EXAMINATION
CBC
RBS, LFT
RFT
Sr.ELECTROLYTES
ECG
HIV
INVESTIGATIONS
OBSERVATIONS
INVESTIGATION OBSERVED VALUE NORMAL VALUE
Hb (g/dl) 12.8 12.0-16.0
WBC (CELLS/Ul) 11,800 4500-10500
Polymorphs% 80% 40-70
Basophiles% 00 0-1
Eosinophiles% 02 0-5
Lymphocytes% 15 20-40
Monocytes% 00 0-7
RBC (mill/uL) 3.97 4,2-5.4
PLT (Cells/Cumm) 2,37,000 1,50,000-4,50,000
IVESTIGATION OBSERVED VALUE NORMAL RANGE
Sr. Urea 19.0 mg/dl 15-30 mg/dl
Sr. creteatinine 0.6 mg/dl 0.6-1.2 mg/dl
ALT 10.0 U/L 6-38 U/L
AST 24.0 U/L 6-40 U/L
ALP 35-140 U/L 323.0 U/L
TOTAL BILIRUBIN O.6 mg/dl 0.2-1.o mg/dl
Direct
Indirect
0.2
0.4
1- 0.4 mg%
1 – 0.6 mg%
RBS 180.0 60-140 mg/dl
ECG: SINUS TACHYCARDIA
HIV : NEGETIVE
BRAND NAME GENERIC NAME DOSE &
ROUTE
FREQUENCY DAY
1
2 3 4 5 6
Inj.dexona Dexamethasone 2cc iv 1-0-1 y y y ______ stop ______
inj.Augmentin Amoxicillin+ Clavunalic
acid
625 mg 1-0-1 y y y y y STOP
Inj.NS withMVI Multi vitamin 1ampule in
100ml ns
0-1-0 y y y y y y
Propygenta cream Clobetasol 0.5%
Salycylic acid0.3%
Q.S
topical
0-0-1 Y Y Y Y STOP
Kenocort cream Triamcinolone Q.S Mucosal 1-1-1 y y y y y y
Tab. Phosmid Cyclophopsphamide 50 mg
P/O
1-0-0 y y y y
Gentian violet solution Gentian violet Q.S 1-0-1 Y Y y y
Inj.Avil Pheniramine maleate 1cc IV 0-0-1Y Y Y Y
Inj. Dexona Dexamethasone 2cc-0-1cc
iv
1-0-1 y y STOP
Inj.Actrapid H.Insulin Acc.S.C S/C As Needed y y SKIP
Inj.Zulig Tigecycline 50mg iv 1-0-1 y
Inj.Dexona Dexamethasone 2cc Iv 1-0-0 y
TREATMENT CHART
PEMPHIGUS VULGARIS
FINAL DIAGNOSIS
The patient was followed up till discharge and her stay was comfortable.
Soon after starting Dexamehasone, The parient developed Hyperglycemia which was treated by Insulin according to
Sliding scale, She also experienced Aloepecia which was due to Cyclophosphamide,
The patient was counselled to have plenty of water post ingestion of Cyclophosphamide to reduce the severity.
Follow up
CARE PLAN
SUBJECTIVE EVIDENCE
FLUID FILLED LESION OVER TRUNK,AND ON UPPER EXTRMITIES WHICH WERE
SPREADING
THE BULLAE LATER RUPTURED TO FORM EROSIONS
ASSOCIATED WITH A FEW CRUSTED LESIONS OVER BODY
EROSION + OVER MOUTH AND PALATE
RBS- 180 Mg/dl
W.B.C -11,800
Sr.Na+ - 128.0
Multiple bulla + all over body
Immuno fluroscence : +ve for Pemphigus vulgaris
OBJECTIVE EVIDENCE
Based on Subjective and objective evidence, the patient is diagnosed to be having
“PEMPHIGUS VULGARIS”
ASSESSMENT
GOALS:
1) Relieve burning sensation
2) Prevent further bulla formation
3) Provide symptomatic relief
4) Monitor for any adverse events
5) Avoid further sepsis
CARE PLAN
RBS
NIKOLSKYS SIGN
NEW BULLA
ELECTROLYTES
DIFFRENTIALS
Monitoring Parameters
TWO ADVERSE DRUG REACTIONS WERE FOUND
1) DEXAMETHASONE INDUCED HYPERGYCEMIA
MANAGED BY DOSE ADJUSTMENT AND INSULIN THERAPY
PHARMACIST INTERVENTION
2) CYCLOPHOSPHAMIDE INDUCED ALOPECIA
DAY 1 DAY 2 DAY 3
NO DRUG-DRUG INTERACTIONS FOUND
NO MAJOR INTERVENTIONS NEEDED
PHARMACOECONOMIC MANAGEMENT OF TIGICYCLINE DONE.
ABOUT DRUGS
CYCLOPHOSPHAMIDE INDUCED ALOPECIA IS REVERSIBLE,
USE GLOVES WHILE APLLYING GENTIAN VIOLET
KENOCORT ISNT HARMFUL EVEN IF SWALLOWED
IN CASE OF ANY ADVERSE EVENT, NOTIFY THE NURSR/ PYSICIAN/PHARMACIST IMMIDIATELY
ABOUT DISEASE:
PV IS AN AUTOIMMUNE DISORDER WHICH ARISES DUE TO INTERNAL LACK OF IMMUNO IDENDTIFICATION.
IT IS NOT ANY KIND OF PUNISHMENT FOR DEEDS
THE DISEASE IS MANAGABLE WITH MAXIMUM RESTORATION OF ACTIVITY
UNFORTUNATELY THERE IS NO PERMANENT CURE>
PATIENT COUNSELING
MINIMIZE SUN EXPOSURE/ USE SUNSCREEN IF NECESSARY
AVOID GARLIC, LEEKS AND OTHER IMMUNO STIMULANTS
TAKE PLENATY OF WATER WHILE USING CYCLOPHOPHAMIDE
USE SOFT CLOTHING
PLANTAIN LEAVES CAN BE USED INDTEAD OF BEDCOVERS
LOOK FOR BED SORES
MILD EXERCISE IS DESIRABLE
LIFE STYLE MODIFICATIONS
ANY QUESTIONS???
THANK YOU!!!

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Pemphigus vulgaris

  • 1. PEMPHIGUS VULGARIS A CASE PRESENTATION BY, ZAHED ULLA KHAN, Pharm.D Intern, H.S.K College of Pharmacy SNMC & H.S.K Hospital and research centre, Bagalkote.
  • 2. AUTOIMMUNE VESCICULOBULLOUS DERMATOLOGICAL MANIFESTATION AFFECTIING EPIDERMIS CHARECTERIZED BY ACANTHOLYSIS INTRODUCTION
  • 3. AUTOIMMUNE ACCOUNTS FOR 70% OF PEMPHIGUS SPECTRUM DUE TO IGG ANTIBODIES ALL GENDERS EQUALLY AFFECTED MEAN AGE IS 30-45 Years RARELY SEEN IN CHILDERN RARELY DRUG INDUCED (MAY BE DUE TO PENICILLIN, ACE INHIBITORS) JEWS, INDIANS,MEDITERANIAN POPULATION INCIDENCE RATE: 0.5-3.3/Yr, PER 100,000 POPULATION (1) AETIOLOGY AND EPIDEMIOLOGY
  • 4. GENETIC PREDISPOSITION HERPES INFECTION UNDERLAYING AUTO IMMUNE DISORDER DRUG SENSITIVITY (RARE) RADIATION RISK FACTORS
  • 5. PATHOPHYSIOLOGY RUPTURE OF JUNCTIONS RELEASE OF CELLULAR FLUID FORMATION OF BULLA RUPTURE LEADING TO EROSION SEPSIS
  • 6. CUTANEOUS EXAMINATION ( NIKOLOSKY’S AND INDIRECT NIKOLOSKY’S) BIOPSY TZANK SMEAR IMMUNOFLUROSCENCE DIAGNOSIS
  • 7. Demiographic Data Name: Mrs. RSG Age: 36 Sex :F Dept. SKIN Unit : A DOA: 6/01/2022 DOD: 27/01/2022 CASE PRESENTATION:
  • 8. C/O FLUID FILLED LESION SINCE 1 YEAR, INSOIDIOUS AND PROGRESSIVE REASON FOR ADMISSION
  • 9. NOT K/C/O HTn, DM, TB, EPILEPSY PAST MEDICAL HISTORY
  • 10. THE PATIENT WAS APPARENTLY ALRIGHT ONE YEAR AGO, THEN DEVELOPED FLUID FILLED LESION OVER TRUNK,AND ON UPPER EXTRMITIES WHICH WERE SPREADING THE BULLAE LATER RUPTURED TO FORM EROSIONS ASSOCIATED WITH A FEW CRUSTED LESIONS OVER BODY EROSION + OVER MOUTH AND PALATE NO Hx OF IBR, DRUG INTAKE, PHOTOSENSITIVITY, JOINT PAIN, WEIGHTLOSS, RASH. HISTORY OF PRESENT ILLNESS
  • 12. Moderately built and nourished Conscious and oriented PICCLE : -ve B.P – 120/80 mmhg P.R - 80 bpm GENERAL PHYSICAL EXAMINATION
  • 14. MULTIPLE EROSIONS +OVER TRUNK, EXTREMITIES, BACK, FACE, AND A FEW LESIONS WERE WITH THICK CRUST. MULTIPLE EROSION + ORAL MUCOSA NAIL, HAIR, GENITAL MUCOSA : NORMAL. CUTANEOUS EXAMINATION
  • 16. OBSERVATIONS INVESTIGATION OBSERVED VALUE NORMAL VALUE Hb (g/dl) 12.8 12.0-16.0 WBC (CELLS/Ul) 11,800 4500-10500 Polymorphs% 80% 40-70 Basophiles% 00 0-1 Eosinophiles% 02 0-5 Lymphocytes% 15 20-40 Monocytes% 00 0-7 RBC (mill/uL) 3.97 4,2-5.4 PLT (Cells/Cumm) 2,37,000 1,50,000-4,50,000
  • 17. IVESTIGATION OBSERVED VALUE NORMAL RANGE Sr. Urea 19.0 mg/dl 15-30 mg/dl Sr. creteatinine 0.6 mg/dl 0.6-1.2 mg/dl ALT 10.0 U/L 6-38 U/L AST 24.0 U/L 6-40 U/L ALP 35-140 U/L 323.0 U/L TOTAL BILIRUBIN O.6 mg/dl 0.2-1.o mg/dl Direct Indirect 0.2 0.4 1- 0.4 mg% 1 – 0.6 mg% RBS 180.0 60-140 mg/dl
  • 19. BRAND NAME GENERIC NAME DOSE & ROUTE FREQUENCY DAY 1 2 3 4 5 6 Inj.dexona Dexamethasone 2cc iv 1-0-1 y y y ______ stop ______ inj.Augmentin Amoxicillin+ Clavunalic acid 625 mg 1-0-1 y y y y y STOP Inj.NS withMVI Multi vitamin 1ampule in 100ml ns 0-1-0 y y y y y y Propygenta cream Clobetasol 0.5% Salycylic acid0.3% Q.S topical 0-0-1 Y Y Y Y STOP Kenocort cream Triamcinolone Q.S Mucosal 1-1-1 y y y y y y Tab. Phosmid Cyclophopsphamide 50 mg P/O 1-0-0 y y y y Gentian violet solution Gentian violet Q.S 1-0-1 Y Y y y Inj.Avil Pheniramine maleate 1cc IV 0-0-1Y Y Y Y Inj. Dexona Dexamethasone 2cc-0-1cc iv 1-0-1 y y STOP Inj.Actrapid H.Insulin Acc.S.C S/C As Needed y y SKIP Inj.Zulig Tigecycline 50mg iv 1-0-1 y Inj.Dexona Dexamethasone 2cc Iv 1-0-0 y TREATMENT CHART
  • 21. The patient was followed up till discharge and her stay was comfortable. Soon after starting Dexamehasone, The parient developed Hyperglycemia which was treated by Insulin according to Sliding scale, She also experienced Aloepecia which was due to Cyclophosphamide, The patient was counselled to have plenty of water post ingestion of Cyclophosphamide to reduce the severity. Follow up
  • 23. SUBJECTIVE EVIDENCE FLUID FILLED LESION OVER TRUNK,AND ON UPPER EXTRMITIES WHICH WERE SPREADING THE BULLAE LATER RUPTURED TO FORM EROSIONS ASSOCIATED WITH A FEW CRUSTED LESIONS OVER BODY EROSION + OVER MOUTH AND PALATE
  • 24. RBS- 180 Mg/dl W.B.C -11,800 Sr.Na+ - 128.0 Multiple bulla + all over body Immuno fluroscence : +ve for Pemphigus vulgaris OBJECTIVE EVIDENCE
  • 25. Based on Subjective and objective evidence, the patient is diagnosed to be having “PEMPHIGUS VULGARIS” ASSESSMENT
  • 26. GOALS: 1) Relieve burning sensation 2) Prevent further bulla formation 3) Provide symptomatic relief 4) Monitor for any adverse events 5) Avoid further sepsis CARE PLAN
  • 28. TWO ADVERSE DRUG REACTIONS WERE FOUND 1) DEXAMETHASONE INDUCED HYPERGYCEMIA MANAGED BY DOSE ADJUSTMENT AND INSULIN THERAPY PHARMACIST INTERVENTION
  • 29. 2) CYCLOPHOSPHAMIDE INDUCED ALOPECIA DAY 1 DAY 2 DAY 3
  • 30. NO DRUG-DRUG INTERACTIONS FOUND NO MAJOR INTERVENTIONS NEEDED PHARMACOECONOMIC MANAGEMENT OF TIGICYCLINE DONE.
  • 31. ABOUT DRUGS CYCLOPHOSPHAMIDE INDUCED ALOPECIA IS REVERSIBLE, USE GLOVES WHILE APLLYING GENTIAN VIOLET KENOCORT ISNT HARMFUL EVEN IF SWALLOWED IN CASE OF ANY ADVERSE EVENT, NOTIFY THE NURSR/ PYSICIAN/PHARMACIST IMMIDIATELY ABOUT DISEASE: PV IS AN AUTOIMMUNE DISORDER WHICH ARISES DUE TO INTERNAL LACK OF IMMUNO IDENDTIFICATION. IT IS NOT ANY KIND OF PUNISHMENT FOR DEEDS THE DISEASE IS MANAGABLE WITH MAXIMUM RESTORATION OF ACTIVITY UNFORTUNATELY THERE IS NO PERMANENT CURE> PATIENT COUNSELING
  • 32. MINIMIZE SUN EXPOSURE/ USE SUNSCREEN IF NECESSARY AVOID GARLIC, LEEKS AND OTHER IMMUNO STIMULANTS TAKE PLENATY OF WATER WHILE USING CYCLOPHOPHAMIDE USE SOFT CLOTHING PLANTAIN LEAVES CAN BE USED INDTEAD OF BEDCOVERS LOOK FOR BED SORES MILD EXERCISE IS DESIRABLE LIFE STYLE MODIFICATIONS

Editor's Notes

  1. 1. Arpita R, Monica A, Venkatesh N, Atul S, Varun M. Oral Pemphigus Vulgaris: Case Report. Ethiop J Health Sci. 2015 Oct;25(4):367-72.