Pemphigus vulgaris

Reference: http://emedicine.medscape.com/article/1064187-overvie
Pemphigus vulgaris is an autoimmune! intraepithelial! "listering disease affecting the s#in and mucous
mem"ranes. $t is mediated "% circulating autoanti"odies directed against #eratinoc%te cell surfaces. & potentiall%
life-threatening disease! it has a mortalit% rate of appro'imatel% (-1().[1]
Signs and symptoms
*ucous mem"ranes
*ucous mem"ranes of the oral cavit% are involved in almost all patients ith pemphigus vulgaris
Patients ma% have ill-defined! irregularl% shaped! gingival! "uccal! or palatine erosions! hich are painful and
slo to heal
$ntact "ullae are rare in the mouth
+rosions ma% "e seen on an% part of the oral cavit%! and the% ma% spread to involve the lar%n'! ith
su"se,uent hoarseness
$n -uvenile pemphigus vulgaris! stomatitis is the presenting complaint in more than (0) of cases
.ther mucosal surfaces ma% "e involved! including the con-unctiva![2]esophagus /causes od%nophagia
and/or d%sphagia0![3] la"ia! vagina! cervi'! vulva![4] penis! urethra! nasal mucosa! and anus
1#in
Primar% lesion of pemphigus vulgaris is a flaccid "lister filled ith clear fluid that arises on health% s#in or on
an er%thematous "ase
2listers are fragile and ma% rupture! producing painful erosions /the most common s#in presentation0
3ails
&cute or chronic paron%chia! su"ungual hematomas! and nail d%strophies affecting one or several fingers or toes
have "een reported ith pemphigus vulgaris.[5, 6]
4egetating pemphigus vulgaris
5esions in s#in folds readil% form vegetating granulations. $n some patients! erosions tend to develop e'cessive
granulation tissue and crusting6 these individuals displa% more vegetating lesions.
1ee Clinical Presentation for more detail.
Diagnosis
5a"orator% studies include the folloing:
7istopatholog%: 8emonstrates an intradermal "lister6 the earliest changes consist of intercellular edema ith
loss of intercellular attachments in the "asal la%er
8irect immunofluorescence /8$90: .n normal-appearing perilesional s#in[7] 6 demonstrates in vivo deposits of
anti"odies and other immunoreactants! such as complement
$ndirect immunofluorescence /$8$90: $f 8$9 results are positive6 circulating intercellular anti"odies are detected
using $8$9 in 80-:0) of patients ith pemphigus vulgaris
1ee Workup for more detail.
Management
;he aim of pharmacologic therap% for pemphigus vulgaris is to reduce inflammator% response and autoanti"od%
production. *edications used in the disease<s treatment include the folloing:
=orticosteroids: 8iscourage the inflammator% process "% inhi"iting specific c%to#ine production
$mmunosuppressants: 1hould "e considered earl% in the course of disease as steroid-sparing agents