pemphigus -2

8/13/2019 pemphigus -2

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MORBIDITY REVIEW

(DERMATOLOGY)

RizwanUllah Khan

PGY -1

Internal Medicine


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PATIENTS PROFILE

Name: P.Q.R.S.

Age: 23 years

Gender: Woman Residence: Gujar Khan

Marital status: Unmarried

D.O.A. 05/01/2011


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PRESENTING COMPLAINTS

Oral ulcers 6 months

Difficulty in swallowing 4 months

Lesions over the trunk 2 months


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HISTORY OF PRESENT ILLNESS Oral ulcers 6 months

Started after taking some medications for fever by a localdoctor

Very painful

Affected the gingival, buccal and palatine region

Gradually started increasing in quantity

Associated with Difficulty in swallowing

Hoarseness of voice


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HISTORY OF PRESENT ILLNESS Difficulty in swallowing 4 months

Initially to solids

Gradually to liquids

Associated with pain in swallowing


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HISTORY OF PRESENT ILLNESS Lesions over the trunk 2 months

Started on the chest moved over towards the abdomenand the upper back

Flaccid skin bullae

Keeps on increasing in size and then oozes out fluid

Affected skin is painful but not itchy


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SOCIAL HISTORY Unmarried, non smoker, non addict, moderate socio-

economic status

PAST HISTORY Medication intake for fever a month before the symptoms

startedFAMILY HISTORY

No history of similar complaints in the family. Non

significant otherwise

DRUG HISTORY Prednisolone 20 mg QD PO 4 months

Folic acid 5 mg QD PO 4 months


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GENERAL PHYSICAL EXAMINATION

A young weak, emaciated woman lying on bed in painwith blisters extending to the upper chest and neckregion

Vitals Pulse: 94 bpm

Resp. Rate: 20 / min

B.P. 130/70

Weight 38 kgs

Afebrile


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Others

Pallor +ve

Lymph adenopathy +ve

Koilonychia +ve

Clubbing

Cyanosis

Jaundice NIL

Edema


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SYSTEMIC EXAMINATION

Abdomen

Soft, non tender, bowel sounds +ve

Chest

Clear bilaterally

CVS

S1 + S2 + 0 CNS

Normal


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LOCAL EXAMINATION

Flaccid skin bullae

Large in size and confluent

Oozing watery fluid Non tender

Associated with granulations and crusting in the neck

and the arm pits region


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DIFFERENTIAL DIAGNOSIS

Pemphigus Vulgaris

Paraneoplastic pemphigus (dysphagia secondary tostricture CA)

Drug reaction


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INVESTIGATIONS

Blood Complete Picture Hemoglobin 9.7 gm/dl

TLC 8.6 x 10^9/l

DLC Neutrophils 70.3% Lymphocytes 28.1%

ESR 15

Peripheral Blood smear Urine Routine Examination Normal

Liver Function Tests

Urea / Electrolytes


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INITIAL MANAGEMENT

Tab Prednisolone 20 mg Q6H PO

Cap Esomeprazole 40 mg QD PO

Cream Clobetasol BID local application Enzichlor MW QID local application

Admit to floor


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MANAGEMENT ON FLOOR


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Day 1

Initial management as Emergency Room plan

Day 2

Upper GI endoscopy carried out Esophagitis - II loweresophagus

Day 3

Rheumatology was consulted

Advised for high dose CS or adjuvant immunosuppressants aspatient was showing no improvement on oral steroids


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Day 3

Patient started on I/V Methylprednisolone 40 mg Q12H andTab Azathioprine 50 mg BID

Skin biopsy performed

Day 4-6

Patient started responding to I/V steroids and Oralimmunosuppressants

Oral lesions improving, skin blisters healing, toleratingorally


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Day 7

Skin biopsy revealed Acantholytic keratinocytes. Epidermis showed acanthosis,

parakeratosis and hyperkeratosis. Lymphocytic infiltrate aroundthe vesicle.

Diagnosis: Pemphigus vulgaris

Day 8

Dermatology planned for discharge


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MEDICATIONS ON DISCHARGE

Tab Prednisolone 20 mg QD PO

Tab Azathioprine 50 mg BID PO

Tab Esomeprazole 40 mg QD PO


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PEMPHIGUS VULGARIS


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PEMPHIGUS VULGARIS

Circulating IgG autoantibodies to antigens on thesurface of keratinocytes

Bind to transmembrane desmosomal cadherins ofkeratinocytes (desmogleins III)

Loss of cell cell adhesionacantholysis +superficial bullae in the epidermal layer

May occur with or without complement activation

If activated Release of inflammatory mediatorsand T cell activation


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http://teachingdermatopathology.blogspot.com/2009/12/15-vesiculobullous-pattern.html


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PEMPHIGUS VULGARIS

Accounts for 70 % of cases of pemphigus Affects women more than men

Average age of onset between 4th6thdecade

Results from an interaction between the hosts geneticfactors and environmental triggering factors Drugs

Diet UV

Viruses

ChemicalsGuidelines for the management of pemphigus vulgaris: Harman K. E., Albert S., Black M. M.; British Journal

o Dermatolo 2003


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PEMPHIGUS VULGARIS

Presentation

Initial lesions in the oral cavity

Skin involvement after around 4 months

Pain, burning and itching

Small minority presents with initial cutaneous lesions


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PEMPHIGUS VULGARIS Diagnosis

Histopathology Suprabasal acantholysis and blister

formation

Direct immunoflourescence(clinically uninvolved skin) Deposition of IgG in the intercellular

spaces of epidermis

ELISA For Dsg I and Dsg III antibodies

Pemphigus vulgaris::a 11 year review; Chumorova N, Svecova D; Bratisk Lek Listy 2009


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1. Suprabasal bullae2. Superficial perivascular inflammatory infiltrate

3. Tombstoning of basal keratinocytes

4. Normal stratum corneum

5. Acantholysis


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PEMPHIGUS VULGARIS

Treatment strategy Remission induction

Maintenance treatment

Treatment withdrawal

Treatment modalities Systemic corticosteroids

Adjuvant steroid sparing medications

Treatment success 38%, 50% and 75% achieved remission 3, 5 and 10 years

from diagnosis

Guidelines for the management of pemphigus vulgaris: Harman K. E., Albert S., Black M. M.; British Journalof Dermatology 2003


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Patient re-presented to the ER on 13/ 4/ 2011


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Pus filled lesions 2 weeks neck, face, chest, back and abdomen

Legs and arms werent involved

Pussy discharge was coming out of it

Oral ulcers 2 weeks

Severe pain

decreased oral intake

PRESENTING COMPLAINTS


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PRESENTING COMPLAINTS Increased frequency of micturition 1 week

No burning or other complaints

Conjunctival injection 1 week

Increased production of tears from eyes

Fever 1 week

Chills and rigors, non documented


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GENERAL PHYSICAL EXAMINATION

A young weak, emaciated woman lying on bed in painwith blisters on the neck, chest, back and abdomen

Vitals

Pulse: 120 bpm

Resp. Rate: 26/ min

B.P. 110/70

Weight 38 kgs

Temperature 100 F


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Others

Pallor +ve

Lymph adenopathy +ve

Koilonychia +ve

Clubbing

Cyanosis

Jaundice NIL

Edema


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SYSTEMIC EXAMINATION

Abdomen Soft, non tender, bowel sounds +ve

Chest Clear bilaterally

CVS S1 + S2 + 0

CNS

Normal Eye

B/L lid rash, Epiphora, difficulty in eye movement, can countfingers from near and far


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LOCAL EXAMINATION

Skin

Scaly lesions with granulations on the neck, chest and

abdomen Extending towards the arm pits

Oozing pussy discharge with foul smell

Tender but non pruritic


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INVESTIGATIONS Blood Complete Picture

Hemoglobin 13.56 gm/dl TLC 10.5 x 10^9/l ESR 30

Urine R/E Turbid Bacteria +ve Leucocyte esterases +ve

Urine C/S Sent Pus C/S Sent Eye discharge C/S Sent Urea & Electrolytes Normal Liver Function Tests Normal


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INITIAL MANAGEMENT Admitted to the floor

Tab Prednisolone 20 mg BID PO

Tab Azathioprine 50 mg BID PO

Inj Cefuroxime 1000 mg BID IV

Cream Gentamycin Betamethasone Local application

Gel Salicylic acidLocal application


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RE-ADMISSION

Day 1 Eye consult

Ofloxacin eye drops

Genteal eye drops

Day 2 -7

Patient didnt respond to corticosteroids

Tab Azathioprine D/C

Tab Prednisolone D/C

Started on Tab Betamethasone 5 mg BID

Started on local application KMnO4


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RE-ADMISSION Day 8-14

Internal Medicine, Rheumatology, Fellow dermatology,Plastic Surgery was consulted

Treatment on the lines of 1stdegree burns as she was oozingfluids from 50 % the total body surface area.

Local application of Provate G + Bactigrass

Continued broad spectrum antibiotic coverage Repeat skin biopsy was carried out

Parenteral nutrition started


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RE-ADMISSION Labs

WBC 134001380015800

Urine C/S showed Mixed insignificant Blood C/S showed MSSA

Pus C/S showed Echinococcus spp

Direct immunofluorescence disgnosed PV

ANA/ AMA/ ASMA Negative


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Antibiotics were adjusted according to sensitivity

Local application of Provate G and KMnO4 bath

Oral and skin lesions started improving

Fever started subsiding

Started tolerating orally

Developed anal fissure relieved by topical GTN Developed low HbPRBC x 2 transfusion

Stool for Occult blood +veRx as steriod induced

gastritis


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Conjunctival injection didnt improve and the patient developedcorneal opacity Keratitis

Pus C/S (eye swab) Pseudomonas aeruginosa Repeat eye consult

Ciprofloxacin PO x 7 days Vigamox Eye drops Tobrex Eye ointment Optoflow Eye ointment

Continued treatment otherwise Patient was showing improvement WBC decreased to 14000

PO Betamethasone decreased to 3.5 mg BID


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RE-ADMISSION Panel didnt allow more coverage and the patient couldnt

afford the treatment herself so was discharged and was lostto follow up

Medications on discharge Tab Betamethasone on tapering dosages

Eye medications

Local applications Oral care agents

Treatment for constipation and anal fissure


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PEMPHIGUS VULGARIS Mortality

Before administration of steroids 90 % 75 % in first year of onset

Morbidity Secondary to steroids

Osteopenia/ osteoporosis

Hyperlipoproteinemia

Cataract Iatrogenic Cushings

Herpes zoster/ Herpetic keratitis

Iatrogenic diabetes

Increased blood pressures

Secondary to CS sparing agents

Deranged LFTs & RFTs

Acute BM suppression

Fungal infections Myopathy


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Br J Ophthalmol 2001;85


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