Penyakit Dermatology Diperantara Imun

Penyakit Dermatology

diperantarai imunCitra Cahyarini

Bagian Ilmu Kesehatan Kulit dan Kelamin FK Universitas YARSI

Blok Imun

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)

A.D may divided into three stages, namely :

• Infantile ( 2 months – 2 years)

• Childhood ( 2 years – 10 years)

• Adult

Pathogenesis : ???

Complex interaction of skin barrier, genetic, Complex interaction of skin barrier, genetic, environmental, pharmacologic and immunologic factorsenvironmental, pharmacologic and immunologic factors

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 Hertoghe’s 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 Kaposi’s 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. Pruritus2. Typical morphology and distribution3. Tendency toward chronics or chronically relapsing dermatitis4. Personal or family history of atopic diseases (asthma, allergic rhinitis,

AD)

Minor criteria :1. Xerosis / ichthyosis/ hyperlinear palms2. Pityriasis alba3. Keratosis pilaris4. Facial pallor / infraorbital darkening5. Elevated serum IgE6. Keratoconus7. Tendency to non spesific hand eczema8. Tendency to repeat cutaneous infections

Differential diagnosis

• Nummular Dermatitis

• Seborrhoic Dermatitis

• Contact Dermatitis

• Psoriasis

• Scabies

General management

1. In infancy and childhooda. 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 & edemasubacute : plaques of mild erythema,

dry scaleschronic : 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 → erythema-

hyperkeratosis & 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

urticaria

angioedema

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 purpuraTest : 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 examinationSkin : 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 doesn’t 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

BULA SUPRABASAL

Macam-macam letak bula

BULA SUBEPIDERMAL

BULA SUBKORNEAL

BULA INTRAEPIDERMAL

LETAK BULA:

1. Bula Epidermal: Letak pada Epidermis Dinding / atap tipis → mudah pecah Ada Dua: - Subkorneal : di bawah str korneum

- Suprabasal : di atas str basalis

2. Bula Subepidermal: Di bawah epidermis Dinding / atap tebal, tegang → tidak mudah pecah,

tahan lebih lama

MEKANISME TERBENTUKNYA BULA:

1. Toksin epidermolisis:

S. aureus toksin str korneum lepas

(bula subkorneal)

2. Invasi virus herpes:

Sel epidermis →degenerasi hidropik

(vesikel intra epidermal)

3. 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

KLASIFIKASI PENYAKIT BULOSA:

1. P.B. AUTOIMUN:o Pemfigus : vulgaris, vegetans, foliaseus, eritemao Pemfigoid bulosao Pemfigus sikatrikalo Dermatitis herpetiformiso Herpes gestasioniso Dermatosis IgA linier pada dewasa (LAD)o Dermatosis bulosa kronik pada anak (CBD)o Epidermolysis bulosa akuisita (EBA)

2. P.B. NON AUTOIMUN:o Epidermolisis bulosa yang diturunkan o Pemfigus familial jinak (Penyakit Hailey-Hailey)o Penyakit akantolitik non familial

3. P.B. LAIN:o Eritema multiformeo Nekrolisis epidermal toksik (TEN)o Porfiria kutanea tarda (PKT)o Dll

P E M F I 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. Superfisial:

Pemfigus foliaseus Pemfigus eritematosa

2. Suprabasal: Pemfigus vulgaris Pemfigus vegetans

PEMFIGUS VULGARIS: Paling sering dijumpai Berat → ancam kehidupan Terutama orang dewasa ♂ dan ♀ samaKLINIS:

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 : - Definitif → letak bula - Akantolisis

- Spongiosis eosinofilik

2. Tes Tzanck : (+) sel akantolitik3. Tanda Nikolsky : (+)4. Imunofluoresensi :

- Direk : 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 terbagik.i :

- Penyakit ginjal- Penyakit hepar- Gangguan hematologik

Siklofosfamid : Bila azatioprin tidak efektife.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 vulgarisKortikosteroid : dosis < Pemfigus vulgaris →

Imunosupresan(-)Dapson : 100-200 mg/hr

PROGNOSIS:Dapat terjadi remisi permanen

DERMATITIS HERPETIFORMIS:(=DUHRING’S 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 ø 2 – 5 mm, berkelompok, dasar eritemo Urtika, erosi, krustao Bula → jarang

o Sembuh → hipo / hiperpigmentasio Lesi simetris, predileksi :lutut, bokong, punggung

atas, sikuo Sangat gatal / kadang seperti terbakaro DH timbul perlahan / kadang mendadako 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, alkoholTopikal : Kortikosteroid krim → potensi sedangSistemik : - 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 terbelahEosinofil pada lepuh~ Pemfigoid bulosa

IF :Direk : IgG dan C3 pada taut dermo epidermalIndirek : (+) 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