urtikaria melinda
TRANSCRIPT
URTIKARIA & ANGIOEDEMAMelinda Rambing Kong
406148015
Pembimbing :
dr. Eko Krisnarto sp.KK
Urtikaria (hives)• Penyakit yang sering dijumpai 15-25% individu pernah
mengalami urtikaria dalam hidupnya• Karakteristiknya:
• Rekuren• Pruritus• edema kemerahan, berbatas tegas, biasanya pada bagian tengah
lebih pucat (wheals)
• Hampir 40% dengan urtikaria juga mengalami angioedema (pembengkakan yang terjadi di bawah kulit)
Imunologi Urtikaria1. Allergens such as pollens, cross the skin
Allergen
Mast cell
IgE
2.Allergens bind to IgE on mast cell in the tissues just bellow the skin
3.Mast cell mediators are released causing redness, itching and swelling (urticaria)
Urtikaria
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DIAGNOSIS• Anamnesa dan Pemeriksaan Fisikfrekuensi; waktu;
durasi; pola berulang dari lesi; bentuk, ukuran, lokasi, dan distribusi lesi; faktor pencetus (mis: makanan, obat, infeksi, gigitan serangga, stimulasi fisik); respon terhadap terapi sebelumnya, dan riwayat atopi dalam keluarga.
• Diagnostik test: • Skin prick tests (SPTs)• Serum-specific IgE tests
Table 1 Conditions to consider in the differential diagnosis of urticariaUrticarial vasculitis •Lesions are usually painful (rather than pruritic), last >48 hours, and leave
discoloration on the skinSystemic mastocytosis •Rare condition that involves the internal organs (liver, spleen, lymph nodes,
bone marrow), in addition to the skinAtopic dermatitis
• Chronic, highly pruritic inflammatory skin disease• Clinical manifestations vary with age
Bullous pemphigoid • Chronic, autoimmune, blistering skin diseaseErythema multiforme
• Acute, self-limited, skin condition• Considered to be a type IV hypersensitivity reaction to certain infections, medications, and other various triggers
Familial cold autoinflammatory syndrome
• Rare, inherited inflammatory disorder characterized by recurrent episodes of rash, fever/chills, joint pain, and other signs/symptoms of systemic inflammation triggered by exposure to cooling temperatures• Onset usually occurs during infancy and early childhood and persists throughout the patient’s life
Fixed drug eruptions
• Lesions occur from exposure to a particular medication and occur at the same site upon reexposure to the offending medication• Lesions usually blister and leave residual pigmentation
Subacute cutaneous lupus erythematosus
• A non-scarring, photosensitive skin condition• May occur in patients with systemic lupus erythematosus (SLE) and Sjögren syndrome
Pruritic urticarial papules and plaques of pregnancy
• Benign skin condition that usually arises late in the third trimester of a first pregnancy
Muckle-Wells syndrome
• Rare genetic disease that causes hearing loss and recurrent hives• May lead to amyloidosis
Schnitzler’s syndrome with monoclonal IgG kappa gammopathy
• Rare disease characterized by chronic, non-pruritic hives, periodic fever, bone and joint pain, swollen lymph glands and an enlarged spleen and liver
Tatalaksana • Hindari pencetus pada beberapa pasien dengan
urtikaria akut (mis: makanan, obat, lateks, bisa serangga)• Antihistamin • Kortikosteroid pada beberapa pasien dengan urtikaria
berat yang tidak respon terhadap antihistamin• Prednison 40mg/hari selama 7 hari
• Terapi imunosupresif & imunomodulator
TATALAKSANA
Table 2 Antihistamines commonly used and indicated for the treatment of urticaria Usual aduld dose Usual pediadric doseSecond-generation H1-receptor antihistamines (first-line therapy)Cetirizine (Reactine) Desloratadine (Aerius) Fexofenadine (Allegra) Loratadine (Claritin)
10-40 mg daily 5-20 mg daily 120-480 mg daily 10-40 mg daily
5-10 mL (1-2 teaspoons) daily (children’s formulation) 2.5-5 mL (0.5-1.0 teaspoon) daily (children’s formulation) Not currently indicated for children under 12 years of age 5-10 mL (1-2 teaspoons) daily (children’s formulation)
First-generation H1-receptor antihistamines (best used as adjunctive therapy for patients with nocturnal symptoms)Hydroxyzine (Atarax) Diphenhydramine (Benadryl)
Cyproheptadine (Periactin)
Chlorpheniramine (ChorTripolon)Clemastine (Tavist-1)
25-50 mg, three to four times daily 25-50 mg, every -6 hours
4-20 mg daily 4 mg every 4–6 hours 1.34 - 2.68 mg, two to three times daily
Children < 6 years: 30 to 100 mg daily in divided doses 2.5-20 mL (0.5-4 teaspoons) every 4 to 6 hours (depending on age/ weight) 2-4 mg, two to three times daily (depending on age/weight) 1 mg every 4–6 hours 1.34 mg, once or twice daily
Angioedema (tanpa Urtikatia)• angioedema tanpa disertai urtikaria jarang ditemukan, dan
harus diwaspadai ke arah diagnosa alternatif, seperti:• Angioedema herediter • Angioedema didapat• Angioedema idiopatik• Angioedema yang berhubungan dengan ACE inhibitor
Angioedema Herediter (HAE)• Penyakit keturunan yang terjadi akibat
kekurangan inhibitor C1 (suatu protein di dalam darah, bagian dari sistem komplemen yang terlibat dalam sistem kekebalan dan reaksi alergi)
• HAE tipe 1: kadar dan fungsi C1 inhibitor menurun• HAE tipe 2: kadar C1 inhibitor normal, tapi fungsinya
menurun• Biasanya pada remaja dan dewasa
Angioedema Didapat (AAE)• Tipe 1: sindrom defisiensi C1 inhibitor yang paling sering
berhubungan dengan penyakit limfoproliferatif sel-B.• Tipe 2: berhubungan dengan hadirnya autoantibodi yang
langsung melawan molekul C1 inhibitor.• Biasanya pada orang tua (dekade ke 4)
• Secara klinis, HAE dan AAE sama, yaitu ditandai dengan angioedema berulang, tanpa urtikaria atau pruritus, yang paling sering menyerang kulit atau jaringan mukosa dari GI dan traktus respiratori bagian atas.
Angioedema Idiopatik• Penyebabnya tidak diketahui• Mekanismenya tidak jelas• Berdasarkan respon pasien terhadap pengobatan,
beberapa kasus diduga disebabkan oleh aktivasi sel mast yang dimediasi oleh mekanisme IgE.
Angioedema yg berhubungan dengan ACE inhibitor• Lebih sering pada pengguna ACE inhibitor:
• Wanita• Perokok• Keturunan Afrika-Amerika
• Dimediasi bradikinin• Paling sering muncul pada minggu pertama penggunaan
ACE inhibitor
Diagnosis • Secara klinis • Pemeriksaan kadar protein komplemen spesifik: kadar C1
abnormal
Tatalaksana
Thank you