cLass Residents Aban 1390

Slide review and quizzDr Faghihi G.

Case no 1.

A 58 yr old female with recent dysphagia ,halitosis and poor food intake ,referred and her exam of oral cavity showed erosions and candidiasis in mouth

Histopath. and DIF. performed What is Dx ??

Pemphigus vulgaris: in Mouth ,, oral lesions

main treatmentoral prednisolone

high-dose regimen a rapid initial control of disease,

but this regimen did not have any long-term benefit over the

moderate-dose with respect to the relapse or the

complications

Rituximab, a monoclonal anti-CD20 antibody .It is hypothesized ,, agent depletes B-lymphocytes

and removes desmoglein antibodies from the circulation.

a promising therapeutic option for refractory immunobullous diseases

A new therap. / agent

Rituximab

Pemphigus and Biologicals…Weekly iv infusion Rituximab 375mg/m2 is very promising in PV recalcitrant

recalcitrant cases of PV show also a good response to etanercept 50mg/twice weekly s.c. up to 6

weeks..

IV Ig in pemphigus

2g/kg/ cycle (given 3-5 days) ,,for lowering side effects pre - hydration and slow rate of

infusion requiredSide effects: aseptic meningitis ,

thromboemboli ,

D+C+P therapy in PV: • 136mg/ day Dexamethasone in 500 cc Dextrose 5%

slow IV drip in 2 hours for 3 consecutive days and, on second day patient receives 500 mg Cyclophosphamide in same drip and

from the first day counted 28 days for next session .

• Between pulses patient receives 50 mg/day cyclphos..

• When patient is lesion –free,, he receives 6 more pulses .

It is advisable patients who are on cyclophosphamide /

prophylaxis against pneumocystitis carinii pneumonia

A 34 yr old female from 3 months ago complains of intense pruritic papulovesicular lesions especially in exterimities and lower trunk

Case 2.

DX??

The histopath

Dermatitis herpetiformis

DH:Rare, chronic, papulo-

vesicular disease characterized by an

intensely pruritic eruption consisting of various

combinations of symmetrical, erythematous,

papular, vesicular, or bullous lesions. The disease is strongly associated with the presence of HLA-DQ2

antigens.Buttocks are a

common site of lesions in DH

pruritic excoriated

papulovesicular rash on those

parts from several months

ago,

in DH anti-endomysial antibodies(IgA)

auto Ag : epidermal transglutaminase

Herpetiform grouping of lesions in

Dermatitis herpetiformis(DH)

In Dermatitis Herpetiformis

an increased risk for autoimmune diseases and lymphoma,

Gluten free dietand dapsone effective

for skin eruption

In DH:many patients can achieve total resolution of skin disease by diet alone,

DH treatment

Dapsone (initial dosage of 0.5 -2 mg/kg/day) can provide dramatic relief of pruritic symptoms, typically within 24 to 48 hours after beginning therapy.

Baseline CBC (G6PD) level should be checked prior to this medication,

weekly CBC till first month, and monthly checks continued for the next 5 months .

Case.no.3

A 4 yr old boy with recent , generalised

eruption somewhat but,

not severely itchy

Linear IgA B. D. of Childhood

LABD

Both in adults and children annular and circinate plaques can seen

Case No.4A 74-year-old man with Alzheimer was referred for a pruriginous and bullous eruption that had developed four weeks after a boiling water burn on his abdomen The lesions consisted of tense bullae developing at the site of the burn initially, Subsequently, the lesions spread to the arms and legs and there were no mucosal lesions .

Linear deposit of IgG

Dx??

Bullous Pemphigoid After thermal Burnn

Bullous

pemphigoidThe condition is not as serious as Pemphigus

V. ansd there is no racial predisposition.oral lesion may occur

only in minority of patients other mucosae

are not involved

Bullous Pemphigoid ,,buccal bulla

Compare previous slide with :/Pemphigus .V

mucous membrane of the mouth  

Case. 5.

In this case , After hospitalization for sepsis and i.v . i nfusion of vancomycin these mucosal leisons appeared a week later and DIF showed linear deposits./ …/..??

Linear IGA disease due to vancomycin

Painful erosions in this middle age lady from a few months no mucosal lesions,otherwise healthy ,drug responsible for

LABD ,Captopril

a 55-year-old man who, after a 6-month history of enlargement of cervical lymph nodes, presented with multiple painful ulcerations of the oral mucosa and lips and multiple skin erosions on the trunk, extremities,. A lymph node biopsy was revealed B-cell non-Hodgkin lymphoma.

Case no.6

shows the blister within the epidermis, with the basal layer on the floor of the blister and the rest of the epidermis making up the roof of the blister. There are

some inflammatory cells in the blister, mainly

eosinophils with a few lymphocytes.

Paraneoplastic pemphigus

Paraneoplastic pemphigus

IIF of rodent bladder as a convenient and cost-effective method of screening for this syndrome, since bladder epithelium has numerous desmosomes,

but the antigens of pemphigus vulgaris and pemphigus foliaceus are not expressed in this tissue

A case with bronchiolitis oblitrans and conjunctival and oral erosions with PNP

she was died due to Bronchiol/oblit/.

Another severly ill patient with SJS like symptoms and pasinful paronychia with Lymphoma/Assoc./ PNP

Some times targetoid or lichenoid lesions inParaneoplastic pemphigus