Bullous disease

Pemfigus

Pathogenesis : diesease of group of pemfigus are tissue specific

autoimmune condition of skin and mucose in which there are specific IgG
autoantibodies which are directed towards the membrane of keratinocytes.

Clinical picture :

Age: between 50 and 60 but it can be seen in children

Primary lesions are non-tense single bullas which develop on clinily

unchanged skin and mucose. Bullaes are easy to burst, leaving eroded
surfaces which slowly heal. PV is monomorphus (one efloresence bulla) in
which there is the secondary polymorphisam(erosion, crust, pigmentation)

In two thirds of the patients the disese is manifested on mucose of the oral
cavity. Primary they are in oral cavity and they can spread to pharynx and
larynx.

Secondary they can be on conjuctiva, labia, anus, penis.

Predelection sites : trunk, intertrigorous regions, scalp.

After bursting the bullae threre are painfull erosions which spread towards
periphery and they don't have tendency towards spontaneous
epitalization.Erosion is coverd with crust. Changes leave the
pigmentation .

Nikolsky I is characteristic sign in active pemfigus; by lateral preassure

on clinically healty skin there is the deatachment of epidermis near bullae.

Nikolsky II direct preassure on the apex of bullae there is the spreading

of bullae.

Compications

The big painfull erosinos and lead to loss of liqid, proteins, electrolites so
patents are exposed to infections. Changes on mucose of the oral cavity,
phanrynx and esophagus cause the disturbances in nutricion because of
difficult swollowing. So it can can cause undernurshment, kahesia and
malaise. Because of damage of larynx there is difficulty of breathing.

If it is not treated PV outcome is lethal.

Histopathology
Pemfigus is characterized by loss of epidermal cells, burst of intarcellular
bridges and the akantolysis. Before the akantolysis there can be the
epidermal invasion of eosinophils which grouped.

Level of akantolysis and forming bullae in PV is localised suprabalsly in

stratum granulosum.

Test for proving

Tzanck test is for fast proving of akantolytic cells in pemfigus. If test is

positive there are single, circle, akantolytic cells with hyperchomatic
nucleus, citoplasm is in center light and on the edge dark.

DIF test is for proving pathogen autoantibodies. In perilesion and clinically

are deposites of IgG in intracellular spaces of epidermis. In 50 % of
patients there are also C3 deposites. IIF test circulating IgG antibodies
directed towards intracelluar supstance epidermis.

Diganosis : Bullaes are large non-tense, easily burst and spread towards
pheriphery. Nikolsky sign is postive, Tzanck test for proving akantolytic
cells, and histopathological analysis for intarepidermal akantolysis with
akantolytic cells. Diagnosis is comfirmed with presence of reticular,
epidermal, fluroesece deposites of IgG and circulating pemfigus antibodies
in blood.

Differential diagnosis : oral lesions can resemble Morbus Bachet,

erosive lichen planus in which tzanck smear is negative. In generalised
bullous eruption bullous pemfigoid.

Treatment

Local therapy-bathing, opening of the bullaes and application of local

antiseptic colors and local antibiotic creams are important for prevention
of seconadry infection. Oral lesion are tereated with local anestetics.

Systemic therapy:

Corticosterid therapy : predisone 1-1,5 mg/kg/tt

Immunosupresive therapy : azatioprin 2-3 mg/kg/dn and ciklophospoamid

100-200 mg/kg/dn.
Pemfigoid bullous

Pathogenesis : PB is disease in which there are circulating autoantibodies

of IgG class directed antigen epitops localized in hemidesmoses.Target
antigens which bind for circuating autoantibodies are bullous pemfigoid
antigens.

Clincal picture :

Age : after 60.

Predelection sites : lower part of abdomen, inner or outer surfaces of

legs, flexor surfaces of upper extremities.

Morphology

In the begining of the disese on the skin can be erytema and sometimes
urtike. Bullae can develop on normal skin or on erytomatous base. Bullaes
are filled with clear contens and sometimes can hemorragic. In bursting of
bullaes on skin there are erosions which have tendecy to epitelise but not
to spread towards pheripery. On the preiphery of the leson there can be
the appernce of vesicules. Bullaes can apper on the oral mucosa.

Histology: subepidermal akantolysis which is filled with eosinophils and

mixed dermal cell infiltate from eosinophils, neutophils, lymphocytes.

Tests

Dif test in perilesion skin there are linear, tubular, fluoresence deposits of
IgG and C3 along basal membrane.

Iif test for detection of of circulating IgG autoantibodies towards basal

membrane.

Diagnosis : is comfimed with finding of subepidermal akantolysis filled

with eosinophils(histology), dif test and iif test.

Differential diagnosis : erythema multiforme, eyrthema fixum bullosum,

porphyria cutena tarda.

Treatment
Corticosterids predisone 40-60mg/day

Antibiotis minocicline, niacinamid

Dermatitis herpetiformis Duhring

Is chronic papulo-vesicualr disese with asymptomatic or symptomatic

gluten senstive enteroptahty and graular IgA in dermal papile.

Clinical picture :

Age : 2 and 4 decade of life.

Ratio : male :female -2:1

Predelection sites : elbows, knees, gluteus, boder of hair and skin of

forhead, neck sholders.

The disese intensivly puritic.

Morphology : changes are localized simetricaly those are exsudative

pauples and small tense vesicules (bullaes) of herpetifom organisation on
erythematous base. Because of intensive puritis changes are excoriated
and sometimes is hard to find vesicule. Mucosa is not affected.

Histology : subepidermal akantolysis and infiltration of dermal papile with

neutrophils . in older lesion there can be eosinophils. Papilary dermis is
edematous and blood vessels are dilatated.

Tests

DIF linear deposits of IgA and C3

IIF circulating antibodies towads muscle endomesium.

Differntial diagnosis : scabies, ezema, purigo, papular urticaria.

Treatment

Dapson , sulfapyridine