Blok Imun

Penyakit
Dermatology
diperantarai imun
Citra Cahyarini

Bagian Ilmu Kesehatan Kulit dan Kelamin

FK Universitas YARSI

Penyakit Dermatology diperatarai imun

Dematitis
Dermatitis Atopik
Dermatitis Kontak
Urtikaria-angioedema
Penyakit vesikobulosa : Pemfigus vulgaris
Erupsi obat : FDE, Makulopapular

Atopic Dermatitis/ Eczema

Def :
Acute, subacute, or chronic relapsing skin
disorder that usually begins in infancy and is
characterized principally by dry skin and pruritus.
Often associated with personal or family history
of atopy such as allergic rhinitis, asthma, and
atopic dermatitis (AD)

Pathogenesis : ???
Complex interaction of skin barrier, genetic,

environmental, pharmacologic and immunologic factors

A.D may divided into three
stages, namely :

Infantile ( 2 months 2 years)

Childhood ( 2 years 10 years)

Adult

Infantile

Usually begins as an itchy erythema of cheeks followed by

development of vesicle, rupture and produce moist crusted
areas

The eruptions may rapidly extend to other parts of the body,

chiefly the scalp, the neck, the forehead, the wrist and the
extremities

The buttocks and diaper area are often involved

The eruption may become generalized with erythroderma

Infantil
AD

Childhood AD

The lesion to be less exudative, drier, and more papular

The classic locations are the antecubital, and the popliteal spaces, the
wrist, eyelids, and the face and in collarette about the neck

The other area, however, are frequently affected

Itching

There is a decrease in the frequency of sensitization to egg, wheat and

milk, but an increase in sensitization to nonigested substances,
particulary wool, cat hair, dog hair, and pollens

Childhood AD

Adolescent and adult AD

Usually the eruption involves the antecubital and popliteal fossae,

the front and sides of the neck, the forehead and the are about
the eyes
Hands dermatitis occurs more frequently in atopic individuals, and
eczematous lessions of the dorsum are usual
Pruritus : paroxysm, nocturnal, triggered by acute emotional stress
Trigger factors : rough clothing, wool irritation, foods or tension.

Adolescent and Adult AD

Associated features

Cutaneous stigmata : Dennie-Morgan fold, Keratosis pilaris,

and Hertoghes sign
Vascular stigmata : White dermographism
Personality traits : Nervous tension
Ophthamologic abnormalities : cataracts, keratoconus.

Susceptibility to infection
:
S.aureus, generalized Herpes simplex or vaccinia virus
infections to produce Kaposis varicelliform eruption

Immunology : elevated serum IgE, decreased T-supressor

cells, decreased chemotaxis and activations of PMN
leucocyte.

Diagnosis
Hanifin & Rajka , Svenson, SCORAD criterias
Hanifin & Rajka criteria :
Major criteria
1.
Pruritus
2.
Typical morphology and distribution
3.
Tendency toward chronics or chronically relapsing dermatitis
4.
Personal or family history of atopic diseases (asthma, allergic rhinitis,
AD)

Minor criteria :
1.
Xerosis / ichthyosis/ hyperlinear palms
2.
Pityriasis alba
3.
Keratosis pilaris
4.
Facial pallor / infraorbital darkening
5.
Elevated serum IgE
6.
Keratoconus
7.
Tendency to non spesific hand eczema
8.
Tendency to repeat cutaneous infections

Differential diagnosis

Nummular Dermatitis

Seborrhoic Dermatitis

Contact Dermatitis

Psoriasis

Scabies

General management
1.

In infancy and childhood

a.
It should be avoided :

External irritation

Sudden change of temperature, excessive

bathing, insufficient cleanless especially in the
diaper region, local infections
b.

Food elimination ( with special attention)

b.

Antihistamin systemically

c.

Olive oil on absorbent cotton may used with gentle

patting for cleansing to avoide rubbing the affected
patrs. Particular attention should be given the genitals
and buttocks and the diapers should be changed

d.

Weak topical corticosteroid.

2.

In adults :
a.
The emosional stress should be controlled
b.
Avoid extremes cold and heat
c.
Hydrated xerotic skin
d.
Antihistamin
e.
Topical steroid ( be ware of the potentiallity)
f.
Antiobiotics ( if nedded)

Contact Dermatitis (CD)

An exogenous dermatitis which develops as a reaction

of the skin to contact with a foreign substance / an
environmental agent, either a primary irritant ( Irritant
CD) or an allergen (allergic CD)
It may be affected by exposure to UV-light, resulting into
two variant reaction : Photoallergic & Phototoxic CD

Allergic Contact Dermatitis (ACD)

Occur in predisposed individual

Sensitization occurs within a week after contact with a
substance (allergen), but there are no visible skin changes
Subsequent contact with allergen, even in small amounts,
causes an dermatitis
Once established, sensitivity may persists for months, years,
or even a lifetime

Symptom : intense pruritus

Physical exam
acute
: erythema & edema
subacute : plaques of mild erythema,
dry scales
chronic
: plaque of lichenification

Lab

: patch test (+)

PATCH TEST

Irritant Contact Dermatitis

Occure in any individual provided the chemical irritant is

applied in a potent enough concentration for a sufficient
length of time

Inflamation of the skin develops at the site of contact

There is non allergic mechanism involved, the damage

result from direct chemical action

Irritants:
strong irritant severe inflamation at the first
contact
Weak irritants: less toxic substances which require
repeated or prolinged contact to
cause inflamation (detergent, organic
solvents, excessive exposure to water)

Incidence:
The incidence of cases of ICD (each type)
depending mainly on the degree of exposure
and the causative agent

In patients with atopic dermatitis there is a

relatively high incidence of ICD

acute ICD

Symptom :
- subjective : burning, stinging, smarting

Physical exam :
- < 24 h
- erythema vesiculation

* acute : sharply demarcated erythema &

superficial edema

vesicles/ blisters

chronic ICD

Cumulative ICD: slowly after repeated additive

exposure to mild irritan

Symptom : stinging & itching, fissure pain

Physical exam :
dryness chapping erythemahyperkeratosis & scaling fissure & crusting

Treatment

Preventive :
Once the causative agent has been identified, further
contact should be avoided
Topical therapy :
in acute state : wet dressing : Burowi solution 1/20 1/40,
Permanganate 1/10.000, followed by topical steroid.
in chronic state : moderate topical steroid
Systemic therapy :
Antihistamin (severe pruritus) and steroid (severe /
extensive eruption

Contact
Dermatitis

URTICARIA & AGIOEDEMA

Def :
* URTICARIA
is compoused of wheals (transient edematous papules &
plaques, usually pruritic and due to edema of papilary body).
The wheals are superficial, well defined.

* ANGIODEMA
is a large edematous area that involves the dermis and
subcutaneous tissue, is deep and ill defined

Therapy
Antihistamin : H1, H1 + H2
Systemic corticosteroid
Adrenalin inj subcutis/ ephedrin tab

angioede
ma

urticari
a

Vasculitis
A heterogeneous group of clinical synd characterized
by inflammation of blood vessels
The clinical picture is essensially dependent of size
and extent of vessel involvement purpura
Test : diaskopi

Therapy

Systemic corticosteroid

FIXED DRUG ERUPTION

Is an adverse cutaneous reaction to an ingested

drug, characterized by the formation of a solitary, but
at times multiple, plaque, bulla, or erosion;
if the patient is rechalleged with the offending drug,
the FDE occurs repeatedly at the identical site within
hours of ingestion

Most commonly implicated agents :

Antimicrobial agents :
tetracycline, sulfonamide, metonidazole, nystatin

Anti-inflamatory agents :
salicylates, NSAIDs

Psychoactive agents :
barbiturates

Oral contraceptives
Quinine
Phenolphthaline
Food coloring

Physical examination
Skin :
Characteristic : a sharply demarcated maculae, round or oval
in shape, occurring within hours after ingestion drug
Initially : erythema, then dusky red to viplaceous
After healing : dark brown with violet hue post inflamatory
hyperpigmentation
Solitary, may be multiple
Distribution : genital skin, but any site involved

Management

Identify and with hold the offending drug

Noneroded lesions : glukokorticoid topical
Eroded lesions : antibiotic topical
Postinflamatory hyperpigmentation : persist at the site
of an FDE months or years and doesnt respond to
Hq

EXANTHEMATOUS DRUG REACTIONS

Is an adverse hypersensitivity reaction to an ingested

or parenterally administered drug characterized by a
cutaneous eruption that mimics a measles-like viral
exanthem; systemic involvement is minimal

Synonyms : maculopapular drug reaction

Pathogenesis
Exact mechanism unknown, probably delayed
hypersensitivity
Reaction to spesific drug :
Amoxicicilin
Allupurinol
NSAIDs
Carbamazepine
Barbiturates
Phenothiazines
Nitrofurantoin
Isoniazid
Hydantoin derivatives Sulfonamides

Physical examination :
Skin
Maculae/ papules erithem, a few milimeters to 1 cm
Purpura ~ vasculitis
Scaling/ desquamation
Distribution : symmetric, almost always on thrunk and
extremities
Mucos membranes : ananthem on buccal mucosa

Management :

Indications for discontinuation of drug

Symtomatic treatment : oral antihistamine
Glucocorticoid agents : topical or oral or IV
Prevention :
must be aware of his spesific drug hypersensitivity

Penyakit vesikobulosa
Adalah :
Suatu penyakit yang ditandai adanya lepuh
(vesikel-bula) pada kulit

Macam-macam letak bula

BULA SUPRABASAL

BULA SUBEPIDERMAL

BULA SUBKORNEAL

BULA INTRAEPIDERMAL

LETAK BULA:
1.

Bula Epidermal:

2.

Letak pada Epidermis

Dinding / atap tipis mudah pecah
Ada Dua: - Subkorneal : di bawah str korneum
- Suprabasal : di atas str basalis

Bula Subepidermal:

Di bawah epidermis
Dinding / atap tebal, tegang tidak mudah pecah,
tahan lebih lama

MEKANISME TERBENTUKNYA BULA:

1.

Toksin epidermolisis:
S. aureus toksin

2.

str korneum lepas

(bula subkorneal)

Invasi virus herpes:

Sel epidermis degenerasi hidropik
(vesikel intra epidermal)

Reaksi imunologis:

Hipersensitivitas:
Dermatitis kontak edema intersel
(bula intra epidermal)

Autoimun (Autoantibodi):
Sel epidermis substansi semen antar sel larut
epidermis lepas bula intra epidermal
(pemfigus vulgaris)
4.
Membrana basalis rusak
Hubungan antar sel basal dan dermis lepas bula
subepidermal (PB, DH, PKT)
5.
Proses mekanis
3.

KLASIFIKASI PENYAKIT BULOSA:

1.

P.B. AUTOIMUN:
o
Pemfigus : vulgaris, vegetans, foliaseus, eritema
o
Pemfigoid bulosa
o
Pemfigus sikatrikal
o
Dermatitis herpetiformis
o
Herpes gestasionis
o
Dermatosis IgA linier pada dewasa (LAD)
o
Dermatosis bulosa kronik pada anak (CBD)
o
Epidermolysis bulosa akuisita (EBA)

P.B. NON AUTOIMUN:

2.
o
o
o

Epidermolisis bulosa yang diturunkan

Pemfigus familial jinak (Penyakit Hailey-Hailey)
Penyakit akantolitik non familial

P.B. LAIN:

3.
o
o
o
o

Eritema multiforme
Nekrolisis epidermal toksik (TEN)
Porfiria kutanea tarda (PKT)
Dll

PE M FI G U S

Penyakit berlepuh intraepitelial

Autoimun tehadap protein spesifik pada mbr sel
epidermis & desmosom
Subtipe :- Pemfigus vulgaris
- Pemfigus vegetans
- Pemfigus foliaseus
- Pemfigus eritematosus
BERDASARKAN LETAK BULA:
1.

2.

Superfisial:

Pemfigus foliaseus

Pemfigus eritematosa
Suprabasal:

Pemfigus vulgaris

Pemfigus vegetans

PEMFIGUS VULGARIS:
Paling sering dijumpai
Berat ancam kehidupan
Terutama orang dewasa
dan sama
KLINIS:
Vesikel jernih, dasar kulit non eritem
membesar >1cm bula tipis pecah erosi
superfisial krusta
PREDILEKSI:
Mukosa mulut : lesi dini
Kulit kepala, lipat paha, vulva / mukosa vagina

Lesi kronik progresif

Gatal, nyeri, terutama mulut sulit makan
Erosi tanda khas
Bau tak enak

PEMFIGUS VEGETANS:

Varian pemfigus vulgaris

Lesi primer : plakat erosif, hipertrofik, hiperplastik,
verukosa (benjolan) dengan pustula
Predileksi : daerah intertriginosa (lipatan)

PEMFIGUS FOLIASEUS:

Bula lebih superfisial

Prognosis tidak begitu berat
Lesi terutama di punggung

PEMFIGUS ERITEMATOSUS:

Varian pemfigus foliaseus

Gejala ~ seperti Lupus Eritematosus
Lok : wajah & kulit kepala, punggung bagian atas

DIAGNOSIS:
1.

Biopsi

2.

Tes Tzanck
Tanda Nikolsky :
Imunofluoresensi
- Direk

3.
4.

- Definitif letak bula

- Akantolisis
- Spongiosis eosinofilik
: (+) sel akantolitik
(+)
:
: IgG

- Indirek : antibodi beredar dalam darah

5. Laboratorium : non spesifik :

Lekositosis, eosinofilia, kadar serum protein
anemi, LED menurun, kelainan elektrolit
6. Kultur dan tes sensitifitas : bila ada infeksi sek

DIAGNOSIS BANDING:

Pemfigoid bulosa
Pemfigoid sikatrikal
Herpes simplek oral
Erupsi obat bulosa

PENGOBATAN:

Rawat Inap : untuk kasus berat

Terapi cairan dan parenteral : lesi mulut yang berat
Topikal :

Kompres : larutan Burrow 1: 40 3 4 x/hr

Antibiotika
Sistemik:
1.
Antibiotika
: ~ kultur dan tes sensitivitas
2. Kortikosteroid : - paling penting
- pilihan : Prednison

Dosis awal:
Lesi kulit 80-100 mg/hr/dosis terbagi, bila lesi
tetap timbul dosis dinaikkan tiap 4 hari, dapat
sampai 200-300 mg/hari
Lesi mulut saja maks 100 mg/hr
Penurunan dosis:
Bila penyakit membaik & stabil slama 2 minggu
3.

Imunosupresan :
- Mengurangi e.s. KS
- Mengurangi dosis KS
- Diberikan segera setelah penyakit dapat dikontrol

Azatioprin : 1-2 mg/kg BB/hr dosis terbagi

k.i :
- Penyakit ginjal
- Penyakit hepar
- Gangguan hematologik
Siklofosfamid : Bila azatioprin tidak efektif
e.s :
- Sistitis hemoragik
- Fibrosis retroperitoneal
dosis bila penyakit dapat dikontrol & dosis
Prednison 20 mg

PROGNOSIS :

Tanpa terapi mortalitas 90%

Pemfigus foliaseus jarang meninggal
Dengan pengobatan remisi, relaps
Sepsis : o.k. infeksi sekunder
50% meninggal o.k. komplikasi terapi:
- perdarahan gastro intestinal
- gangguan elektrolit

PEMFIGOID BULOSA:

Bula subepidermal tegang tidak mudah pecah

Otoantibodi : anti membrana basalis
Lesi primer : bula tegang, isi jernih, p.u. dasar
tidak eritem
Dapat ditemukan bula hemoragik
Diameter bula : beberapa mm 6 cm
Sembuh tanpa bekas
Lesi tersebar seluruh tubuh
20% kasus disertai lesi oral
pu. asimtomatik, dapat diserta gatal

Tes Tzanck
: (-), Akantolisis (-)
Nikolsky
: (-)
Imunofluoresen :
- Direk
: - IgG btk pita pada taut dermo epidermal
- Komplemen
- Indirek : Antibodi yang beredar dalam darah

TERAPI:
~ Pemfigus vulgaris
Kortikosteroid : dosis < Pemfigus vulgaris
Imunosupresan(-)
Dapson
: 100-200 mg/hr

PROGNOSIS:
Dapat terjadi remisi permanen

DERMATITIS HERPETIFORMIS:
(=DUHRINGS DISEASE)
Penyakit Autoimun : IgA granuler pd papila dermis
Insiden : tidak diketahui
: = 3:2
Timbul : dekade 3
Hubungan dg hipersensitivitas terhadap gluten
(enteropati gluten sensitif)
KLINIS:
o Vesikel 2 5 mm, berkelompok, dasar eritem
o Urtika, erosi, krusta
o Bula jarang

o
o
o
o
o

Sembuh hipo / hiperpigmentasi

Lesi simetris, predileksi :lutut, bokong, punggung
atas, siku
Sangat gatal / kadang seperti terbakar
DH timbul perlahan / kadang mendadak
Tanpa terapi penyakit akan lama

DIAGNOSIS:

Biopsi
: - bula subepidermal
Lekosit p.m.n
IF
: IgA granuler pada dermis bagian atas
Serologis eosinofilia
Radiologik : kelainan di usus halus

DIAGNOSIS BANDING:

Pemfigoid bulosa
Herpes gestasiones
Skabies
Reaksi obat
DKA

TERAPI:
Diit
: Hindari gluten : gandum, alkohol
Topikal : Kortikosteroid krim potensi sedang
Sistemik : - Dapson 200 mg/hr
- Tidak terbentuk lesi baru dosis

HERPES GESTASIONES:

Penyakit otoimun berlepuh yg jarang dijumpai

Khas : Autoantibodi terhadap T.D.E
Pada kehamilan / keganasan trofoblast
Swasirna
Ada hub dg mola hidatidosa / koriokarsinoma
Tidak menular
Tidak ada hub dg herpes simplek/ zooster
Patofisiologi belum jelas

Gambaran Klinis:

Lesi awal papula / plakat / urtika

Sangat gatal
Vesikel bula tegang, pecah krusta
Ukuran lesi beberapa mm cm
Lesi mulai dari abdomen tu umbilikus meluas
seluruh tubuh & ekstremitas, wajah, telapak tangan
& kaki
P.u. membrana mukosa tak terlibat
Timbul pada trimester 2 / 3
Dapat sehari sebelum persalinan
Lesi urtikaria dapat menetap beberapa bulan
Lesi pada bayi (-)

DIAGNOSIS:

Biopsi:
Epidermis terbelah
Eosinofil pada lepuh
~ Pemfigoid bulosa
IF :
Direk
: IgG dan C3 pada taut dermo epidermal
Indirek
: (+) pada beberapa kasus

DIAGNOSIS BANDING:

Pemfigoid bulosa
Eritema multiforme
Dermatitis herpetiformis
PUPPP (Pruritic Urticaria Papule and Plaques of
Pregnancy)

TERAPI:
Ringan:
KS topikal potensi sedang, oles 4 6 x/hr
Antihistamin peroral
Berat:
Prednison 40 mg/hr/oral
P.u. dapat atasi penyakit dengan cepat, kemudian
diturunkan sampai 10 20 mg/hr
Plasmaferesis : bila tidak berespon pada KS
Pengobatan pd bayi baru lahir : Tidak perlu, karena
lesi transien

PROGNOSIS

Sebagian besar kasus sembuh beberapa

minggu setelah persalinan
Eksaserbasi:
- Periode menstruasi
- Penggunaan kontrasepai oral
- Kehamilan berikut

CBDC =
CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD

Jarang dijumpai
Pada masa bayi dan anak ( < 5 tahun)
IgA linier pada taut dermo epidermal
Patogenesis belum diketahui
P.u. didahului infeksi saluran nafas atas

KLINIS:

Vesikel berkelompok
Gatal (+) / (-), kadang hebat
Sembuh
: hipo / hiperpigmentasi
Predileksi
: perioral, genital
50% kasus
: lesi mulut

DIAGNOSIS:

Biopsi
: ~ DH
IF direk : IgA linier pada taut dermo epidermal
IF indirek : otoantibodi anti mbr basalis (80% kasus)

DIAGNOSIS BANDING:

Varisela
Impetigo
Pemfigoid bulosa
Eritema multiforme

TERAPI:

~ DH
Dapson
Sulfapiridin
KS sistemik : sedikit memberi keuntungan

PEMFIGUS VULGARIS

PEMFIGUS ERITEMATOSUS

PEMFIGOID BULOSA

CHRONIC BULLOUS DERMATOSIS OF

CHILDHOOD

CC, juni 2008