CASE STUDY ON PEMPHIGUS VULGARIS

SRM MEDICAL COLLEGE AND RESEARCH CENTRE

PRESENTED BY:
[RA2022281010001]
DEPARTMENT OF PHARMACY PRACTICE
SRM COLLEGE OF PHARMACY, SRMIST
KATTANKULATHUR- 603203
Patient details
Name Age Sex

Mr. S 47 years male

Dept Weight: 85.2 KG

Height: 173 cm
DERMA
 Subjective Evidences
Reason for admission
K/C/O Pemphigus Vulgaris patient on 7th cycle of DCP therapy
Patient developed Fluid filled lesions all over the body 1 and a half years back

Past Medical History

Completed 6 cycles of DCP
No oral lesions
N/K/C/O T2DM, SHTN
H/O Joint pain x 1 year (B/L Hip pain  B/L knee and other joints)
 OBJECTIVE EVIDENCES
GENERAL EXAMINATION:
Post inflammatory hyperpigmentation in the trunk, abdomen, UL,LL
PHYSICAL EXAMINATION:
BP: 120/90mm/hg
PR: 90/min
RR:24/min
SYSTEMATIC EXAMINATION:
CVS: S1S2+
RS: B/L NORMAL VESICULAR BREATH SOUNDS HEARD
CNS: NFND
P/A: PA soft tender
 LABORATORY INVESTIGATION
HAEMATOLOGY D1 D2 D3 D4 NORMAL RANGE
Haemoglobin 13 10.1 8.1 8.2 M-[13-17 g/dL]
F-[12-15 g/dL]
PCV 42 32 26 27 36-46%
MCV 94 67 50 31 83-101fL
MCH 22 22 21 21 27-32 pg
MCHC 31 32 55 70 31.5-34.5 g/dL
WBC 8070 28990 21170 14820 4000-11000 cells/cu.mm
 HAEMATOLOGY D1 D2 D3 D4 NORMAL RANGE
Neutrophils 77 91.1 40-80%
Lymphocytes 15 3.6 20-40%
Monocytes 5.7 2.8 2-10%
Platelets 210000 288000 246000 219900 1,50,000-4,00,000 cells/cu.mm
ESR 5.0 M (0-10mm/hr)
F (0-20mm/hr)
LIVER FUNCTION TEST:

LIVER FUNCTION TEST VALUE NORMAL RANGE

SGOT 16 8.0-40U/L
SGPT 11 5.0-35U/L
ALP 62 40-125U/L
Albumin 4.3 3.5-5.0 g/dL
Globulin 1.7 2.5-3.0 g/dL
RENAL FUNCTION TEST
RENAL FUNCTION TEST D1 D2 D3 D4 NORMAL RANGE
Serum urea 61 67 56 46 15-40mg/dL
Blood Urea Nitrogen 29 31 26 21 7.0-20mg/dL
Serum creatinine 2.0 1.4 1.3 1.5 0.5-1.2 mg/dL

ELECTROLYTES
ELECTROLYTES D1 D2 D3 D4 NORMAL RANGE
Sodium 132 135 136 137 135-150 mmol/L
Potasium 4.0 3.7 3.4 3.7 3.0-5.0 mmol/L
Chloride 98 102 104 105 95-105 mmol/L
Bi-Carbonate 20 23 21 22 22-29 mmol/L
Calculation of GFR
using Cockcroft-
Gault equation
The GFR was found to be 78.65
ml/min/1.73 m2
Final Diagnosis –
Pemphigus
Vulgaris
 MEDICATION CHART:

S.N DRUG GENERIC DOSE ROUTE OF FREQUENCY D1-D3

O NAME NAME ADMINISTRATI
ON

1. T. Prenisolone Prednisolone 5 mg P/O 1-0-0 D1-D6

OD
2. Inj PAN 40 Pantoprazole 5mg IV 1-0-0 D1-D6
OD
3. Tablet MMF Mycophenolat 500mg P/O 1-1-1 D1-D6
e TD
Mofetil
4. C.ABDIFER Vitamin B9 P/O 1-0-0 D1-D6
( folic acid ) OD
5. T. Vit D3 Cholecalciferol 60K IV WEEKLY D1-D6
ONCE
6. T. SHECAL Calcium and 500 mg P/O 0-1-0 D1-D6
cholecalciferol OD
7. FLUCIBERT CREAM Betamethasone 150 mg Topical 0-0-1 D1-D6
+fucsidic acid OD
 Pharmacist Intervention
• DCP Therapy – Dexamethasone – Cyclophosphamide Therapy
DAY 1 Dexamethasone 100mg in 500ml of 5% dextrose IV over 2h
DAY 2 Dexamethasone 100mg + 500mg Cyclophosphamide in 500ml of 5% dextrose IV over 2h
DAY 3 Dexamethasone 100mg in 500ml of 5% dextrose IV over 2h

INTERVENTION : Cyclophosphamide-induced haemorrhagic cystitis

prevention: On day 2 of cyclophosphamide infusion, the patient is
advised to empty the bladder half hourly during infusion until 2 h
after the infusion.

•Bladder toxicity can be reduced by administration of IV mesna

during IV doses of cyclophosphamide. The dose of mesna is
equivalent to cyclophosphamide dose in 5 divided doses over 24 h

REFERENCE: IJDVL – Indian Journal of Dermatology, Venereology & Leprosy

Recent Advances in
Understanding Pemphigus
Journal of Investigative Dermatology - https://doi.org/10.1016/j.jid.2019.11.005
• Pemphigus is a term that includes two major autoantibody-mediated diseases,
pemphigus vulgaris (PV) and pemphigus foliaceus (PF)
• Classical and seminal studies showed that almost all patients with pemphigus
have anti-desmoglein (Dsg) antibodies and anti-Dsg antibodies can cause typical
pemphigus pathology
• In PF, anti-Dsg1 antibodies cause the loss of cell adhesion in the superficial
epidermis, and in PV, anti-Dsg3 or anti-Dsg3 with anti-Dsg1 antibodies cause
blisters deep in the epidermis or mucosal epithelium
• A pathogenic mouse PV IgG binds to the adhesive interface of Dsg3, suggesting
that by interfering directly with Dsg-mediated adhesion, it can cause PV blisters
• Seminal studies have validated anti-CD20 antibody therapy with rituximab as
a very effective therapeutic approach for both PV and PF and it is now
considered the first line therapy.
• The effectiveness of anti-CD20 antibody therapy, which targets B cells but
not long-lived plasma cells, suggests that pemphigus antibodies are produced
by short-lived plasmablasts that require continual renewal by memory B cells
• DURATION OF DCP THERAPY
The duration of infusion of CP according to Pasricha is 2 h and according to Balachandran is 3–4 h. However, half-life is
7 h, and hence, it should be given over maximum 2 h to maintain the maximum concentration over a short-time.
• Prevention of steroid-induced osteoporosis by administration of calcium supplementation (500 mg/day),
Vitamin D (400 IU/day), and bisphosphonate (e.g., alendronate).

• Alternative Therapy: Rituximab (Monoclonal Antibody)

It is the first line therapy for pemphigus vulgaris, it was approved by FDA in 2018. It has lesser mortality rates in
patients with pemphigus vulgaris, as compared to DCP Therapy.
- Rituximab is also given for pemphigus according to the rheumatoid arthritis protocol as 2 doses of 1 g, 2
weeks apart and according to the lymphoma protocol as 375 mg/m2 weekly for 4 weeks.
CONTRAINICATIONS TO KEEP IN MIND
• Patients receive antibiotics especially cephalosporins in view of bacterial skin infection, until the skin
lesions heal, however, cephalosporins aggravate pemphigus
• Advised that antibiotics should be used until the infection clears and not until lesions heal
• REFERENCE: https://doi.org/10.4103/0378-6323.45236
Patient Counselling

Disease based Counselling

Pemphigus vulgaris (PV) is an autoimmune disease that results in blisters on cutaneous and mucosal
surfaces.

Drug based Counselling

• Patient was advised to take T. MMF twice a day on empty stomach, (1 hour before or 2 hours
after a meal)
• Factors that aggravate disease: Sudden withdrawal of systemic corticosteroids, thus patient was
advised not to stop medications without consultation with a physician.
• Black stools, along with constipation is common when taking Iron supplements
• All the drugs should be taken on time, till completion
Patient Counselling
Diet Counselling
• Patient was advised to take foods containing high
iron Levels and vitamin C rich foods at the same
time.
IRON: Leafy greens, Liver, fruits like pomegranate etc
VITAMIN C: Citrus Fruits, papaya, guava
• Patient was also advised to take foods containing Calcium,
as hypocalcaemia is an etiological factor that leads to
psoriasis.
 Patient Counselling
Lifestyle Modification:
• Intervention strategies that promote health such as Yoga, relaxation, visual imagery, meditation, group support,
These forms of intervention, when used as an adjunct to regular pharmacological therapy, produce a significant
clinical benefit by reducing stress.
• Emotional stress may influence the development and exacerbation of disease in 37–78% patients.
• Soaking in warm water with a bath oil or tar solution and use of soft brush softens and lifts the
scales in patients with psoriasis. Bland soaps or soap substitutes should be used while antiseptics
should be avoided because they may irritate the skin. Oatmeal baths and wet dressings relieve
itching