pemphigus

Question 1

diff pemphigus vulvaris and folaceius based on skin lesions and eye acantholysis

Answer 1

■ Aserious, acute or chronic, bullous autoimmune disease o skin and mucous membranes based on acantholysis.
■ Twomajortypes:Pemphigusvulgaris(PV)andpemphigus oliaceus(PF).
■ PV: Flaccid blisters on skin and erosions on mucous membranes. PF: Scaly and crusted skin lesions.
■ PV: Suprabasal acantholysis. PF: Subcorneal acantholysis.
■ IgG autoantibodies to desmogleins, transmembrane desmosomal adhesion molecules.
■ Serious and o ten atal, unless treated with immunosuppressive agents.

Question 2

age onset

gender

Answer 2

AGEOFONSET Fortyto60years; ogoselvagem also occurs in children and young adults.
SEX Equal incidence in males and in emales, but predominance o emales with PF are in
unisia and Colombia.

Question 3

pathogenesis

Answer 3

Anautoimmunedisorder.Losso cell-to-cell adhesion in the epidermis (acantholysis

Occurs as a result o circulating antibodies
o theIgGclass,whichbindtodesmogleins, transmembrane glycoproteins in the desmo- somes, members o the cadherin super amily. Desmosomes hold epidermal cells (keratino- cytes) together. In PV, desmoglein 3 (in some, also desmoglein 1). In PF, only desmoglein 1. Autoantibodies inter ere with calcium-sensitive adhesion unction and thus induce acantholysis

Question 4

positive of what sign

Answer 4

NikolskySign. Dislodgingo normal-appearing epidermis by lateral nger pressure in the vicin- ityo lesions,whichleadstoanerosion.Pressure onbullaleadstolateralextensiono theblister.

Question 5

P LV skin lesio

Answer 5

Pemphigus Vulgaris usually starts in the oral mucosa, but months may elapse be ore skin lesionsoccur.Less requently,theremaybea generalized, acute eruption o bullae rom the beginning. No pruritus but burning and pain in erosions. Pain ul and tender mouth lesions may prevent adequate ood intake. Epistaxis, hoarseness, and dysphagia. Weakness, malaise, and weight loss.
SKINLESIONS Vesiclesandbullaewithserouscon- tent, accid ( abby) (Fig. 6-8), easily ruptured, and weeping (Fig. 6-9), arising on normal skin, randomly scattered, and discrete. Localized (e.g., to mouth or circumscribed skin area) or general- ized with a random pattern. Extensive erosions bleed easily (Fig. 6-10), crusts particularly on scalp. Since blisters rupture so easily, only pain ul erosions in many patients.

Question 6

prediliction

Answer 6

SitesofPredilection. Scalp, ace,chest,axillae, groin, and umbilicus. In bedridden patients, there is extensive involvement o the back

Question 7

P vegetans area of predilectio

Answer 7

PemphigusVegetans(PVeg). APVvariant. Usuallycon nedtointertriginousregions,peri- oral area, neck, and scalp

Question 8

drugs causing drug induced pv

Answer 8

captoprilandD-penicillamine

Question 9

P foliaceus skin lesion

Answer 9

PemphigusFoliaceus. PFhasnomucosal lesionsandstartswithscaly,crustedlesionsonan erythematousbase,initiallyinseborrheicareas. SKIN LESIONS Most commonly on the ace, scalp,upperchest,andabdomen.Scaly,crusted erosionsonanerythematousbase

Question 10

Brazilian pemphigus other name

Answer 10

fogo salvagem

Question 11

pemphigus erythematosus synonym

Answer 11

PemphigusErythematosus(PE). Synonym: Senear-Ushersyndrome.Alocalizedvarianto PF largelycon nedtoseborrheicsites.Erythematous, crusted,anderosivelesionsinthe“butter y”area o the ace, orehead,andpresternalandinter- scapularregions.Mayhaveantinuclearantibodies

Question 12

neonatal pemphigus mode of transmissio

Answer 12

Veryrare,transpla- centaltransmission romdiseasedmother

Question 13

DP

Answer 13

PV: Light microscopy (selectearlysmallbullaor,i notpresent, margin o larger bulla or erosion). Separation o keratinocytes,suprabasally,leadingtosplit

just above the basal cell layer and vesicles con- taining separated, rounded-up (acantholytic) keratinocytes. PF: Super cial orm with acan- tholysis in the granular layer o the epidermis

Question 14

immuno path

Answer 14

IMMUNOPATHOLOGY Direct immuno uores- cence(IF)stainingrevealsIgGando enC3

SERUM Autoantibodies (IgG) detected by indirect IF or ELISA. iter usually correlates withactivityo disease.InPV,autoantibodies against a 130-kDa glycoprotein, desmoglein 3, located in desmosomes o keratinocytes. In PF, autoantibodies to a 160-kDa intercellular (cell sur ace) antigen, desmoglein 1, in desmosomes o keratinocytes.

Question 15

tx

Answer 15

GLUCOCORTICOIDS 2to3mg/kgbodyweight o prednisoneuntilcessationo newblister
ormationanddisappearanceo theNikolsky