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1 대한내과학회지 : 제 94 권제 4 호 Roadmap to final diagnosis 두드러기의분류와진단 아주대학교의과대학알레르기내과학교실 예영민 Urticaria: Classification and Diagnosis Young-Min Ye Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea Urticaria is a common cutaneous disease characterized by recurrent and transient wheals and pruritus, sometimes accompanied angioedema. The classification of urticaria is based on the duration of the disease and whether extrinsic triggers are identified or not. Acute urticaria is usually occurred by specific causes, such as drug, food, and infection, etc. Therefore, acute urticaria can be remitted within 6 weeks just by avoiding the exposure to the causes. However, chronic urticaria defined as repeatedly occurred itchy wheals and/or angioedema for at least 6 weeks, has a significant effect on patients quality of life. Chronic inducible urticaria can be triggered by various physical stimuli including dermographism, delayed pressure, cold, heat, cholinergic stimuli, sunlight, and exercise. Chronic spontaneous urticaria (CSU) is diagnosed when no specific extrinsic cause is identified in the patients. CSU due to autoimmune mechanism accounts for 30-50%, autologous serum skin test and anti-thyroid autoantibody can be evaluated. However, various physical stimuli, emotional or physical stress, drugs, particularly aspirin and non-steroidal anti-inflammatory drugs can exacerbate urticaria in 30-75% of patients with CSU. Allergic diseases and autoimmune diseases are more common in CSU patients than in general populations. To assess the severity of urticaria and to adjust treatment step, urticaria activity score over 7 days, calculated by the number of wheals and the severity of pruritus, is recommended by recent international guidelines. (Korean J Med 2019;94: ) Keywords: Urticaria; Angioedema; Acute urticaria; Chronic urticaria 증 48 세남자가두눈꺼풀의심한부종으로내원하였다. 3 개 례 월전부터거의매일, 특히야간에심한피부가려움과팽진이반복되던중내원당일아침갑자기두눈꺼풀이부어올랐다고한다 (Fig. 1). 피부가려움과팽진은 2개월전부터개 Received: Revised: Accepted: Correspondence to Young-Min Ye, M.D., Ph.D. Department of Allergy and Clinical Immunology, Ajou University School of Medicine, 164 World cup-ro, Yeongtong-gu, Suwon 16499, Korea Tel: , Fax: , ye9007@ajou.ac.kr Copyright c 2019 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

- The Korean Journal of Medicine: Vol. 94, No. 4, 2019 - 인의원에서 cetirizine, fexofenadine을처방받아복용중이며, 가려움은조금나아졌으나팽진이지속되고, 증상이너무심한날은병원에서스테로이드주사도맞았다고한다. 하루중에도여러차례가렵고, 부푸는증상이특별한자극없이나타난다고하였다.

압통과열감이없는전반적인눈꺼풀부종과몸통과팔, 다리에팽진과주변홍반이관찰되었고, 팽진이서로뭉쳐서갯수를헤아릴수없는것도있으며, 가려움이심하여견딜수없을정도라고하였다. 서론주요토의항목 - 두드러기의분류 : 급성 vs. 만성 - 만성두드러기의분류 : 만성자발성두드러기 vs.

2 - The Korean Journal of Medicine: Vol. 94, No. 4, 인의원에서 cetirizine, fexofenadine을처방받아복용중이며, 가려움은조금나아졌으나팽진이지속되고, 증상이너무심한날은병원에서스테로이드주사도맞았다고한다. 하루중에도여러차례가렵고, 부푸는증상이특별한자극없이나타난다고하였다. 알레르기비염이있어서간헐적으로치료받았고, 약물및식품에대한알레르기는없었다. 내원 2 시간전두통으로 ibuprofen을복용한것외에는특별한음식과약물에노출되지않았다. 내원시체온 37.2, 혈압 135/80 mmhg, 호흡수 20회 / 분, 청진상천명음은없었다. 압통과열감이없는전반적인눈꺼풀부종과몸통과팔, 다리에팽진과주변홍반이관찰되었고, 팽진이서로뭉쳐서갯수를헤아릴수없는것도있으며, 가려움이심하여견딜수없을정도라고하였다. 서론주요토의항목 - 두드러기의분류 : 급성 vs. 만성 - 만성두드러기의분류 : 만성자발성두드러기 vs. 만성유발성두드러기 - 두드러기중증도평가 : 항히스타민제치료반응성, 혈관부종동반 두드러기의분류두드러기는갑자기발생하는팽진, 혈관부종을특징하는질환으로전체인구의약 20% 가일생에한번은경험하는것으로알려져있다. 두드러기는팽진, 혈관부종, 피부가려움등특징적인증상의지속기간에따라급성과만성으로분류한다. 팽진이발생한후 6주이내에완전히치료되거나자연소실되는경우를급성두드러기로분류한다. 주로식품, 약물, 감염등에의하여일시적으로나타나는급성두드러기는해당원인이소실되면호전되어더이상증상이나타나지않는다. 반면팽진이 6주이상거의매일나타나는만성두드러기는악화와호전을반복하며그경과를예측할수없어삶의질을떨어뜨린다. 팽진은일시적으로나타나며대부분 24시간이내에소실된다. 팽진이 24시간이상동일부위에지속될경우피부조직검사로두드러기성혈관염을감별하여야한다. 만성두드러기의분류및진단만성두드러기는특별한외부유발요인에의하여반복적으로발생하는만성유발성두드러기와외부유발요인없이저절로또는이유없이반복되는만성자발성 / 특발성두드러기로분류한다 [1]. 만성유발성두드러기는주로한랭, 햇빛, 묘기증, 압박, 운동, 체온상승등다양한물리적자극에노출될때팽진과가려움이발생하고, 자극이없을때는증상이 Figure 1. Periorbital angioedema and wheals on the trunk

3 - Young-Min Ye. Classification and diagnosis of urticaria - 나타나지않는상태가 6주이상지속될때로정의한다 (Fig. 2). 자세한병력청취를통하여확인할수있지만, 객관적인유발시험을통하여확진및진단당시자극에대한역치를평가할수있다 [2]. 만성자발성두드러기는자가반응성또는자가면역성, 가성알레르기, 음식물에대한불내성과드물지만악성종양등유발및악화요인에따라다양한아형으로분류되며, 일부환자에서는여러요인이복합적으로작용하기도한다. 만성자발성두드러기환자에서도한가지또는그이상의다양한물리적자극들이악화요인으로작용할수도있다. 최근연구에서는만성자발성두드러기환자의 76% 에서물리적자극에의한유발성두드러기가동반된것으로보고하였다 [3]. 두드러기의진단은문진과진찰, 의심되는음식, 약물, 물리적인자에대한유발검사를포함한다 (Table 1) [1,4]. 진단절차는환자의병력과두드러기의분류에따라권장되는검사의항목이다르다. 만성두드러기의진단을위하여다른동반질환및물리적두드러기여부를확인하고, 문진을통하여가능한원인을파악해야한다. 말초혈액검사, 적혈구침강속도, C-반응단백검사를통하여감염질환의동반가능성을확인한다. B형및 C형바이러스간염, 단순포진, Ebstein-Barr 바이러스, 기생충등의감염이두드러기의원인이될수있다. 또한갑상선질환의과거력이있는경우자가항체검사도고려의대상이될수있다. 갑상선질환이외에도여러자가면역질환이있는경우두드러기증상이동반될수있으므로문진에서의심되는경우추가검사를시행해볼수있다. 두드러기환자에서자가항체가흔히발견되지만이의임상적연관성이뚜렷하지않으므로모든환자에서자가항체검사를시행하는것은추천되지않는다. 팽진이없이혈관부종만반복적으로나타나는경우유전성혈관부종을의심하여 C4, C1 inhibitor를측정한다. 만성두드러기환자의 30% 이상에서아스피린및진통소염제과민증이동반되므로의심되는경우경구유발검사를시행해볼수있다. Immunoglobulin E (IgE) 또는고친화성 IgE 수용체에대한자가면역반응을확인하기위하여자가혈청피부반응시험을시행할수있다. 두드러기중증도평가두드러기중증도평가는두드러기활성도점수 (urticaria activity score), 두드러기조절점수 (urticaria control test, UCT), Figure 2. Classification and diagnosis of urticaria. NSAIDs, non-steroidal anti-inflammatory drugs; ACEIs, angiotensin-converting enzyme inhibitors; CBC, complete blood count; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein

4 - 대한내과학회지 : 제 94 권제 4 호통권제 689 호 Table 1. Recommended evaluations for the diagnosis of urticaria History taking 1. Frequency, duration, and extrinsic triggers of wheals 2. Diurnal variation 3. Presence of wheals during weekends, travel, occupation, or hobbies 4. Size and distribution of wheals, pruritus or pain 5. Angioedema 6. Family history of urticaria and atopy 7. History of allergic diseases and infections 8. Emotional stress 9. Surgery (implant, symptoms during anesthesia) 10. Combined gastrointestinal symptoms 11. Exercise or other physiological triggers 12. Medication use (NSAIDs, ACEIs, vaccination, hormones, herbal medicines, etc.) 13. Food, association with the menstrual cycle 14. Smoking Classification of urticaria Routine test Extended diagnostic evaluation Spontaneous urticaria Acute Not recommended Not recommended Chronic CBC Infection ESR, CRP Type I hypersensitivity Quit suspected agent Functional autoantibody Thyroid hormones and antibodies Skin tests, including physical tests Pseudoallergenfree diet Tryptase (systemic reactions) Autologous serum skin test Skin biopsy Inducible urticaria Cold Cold provocation and threshold test (ice cube, cold water) CBC, ESR, CRP, cryoproteins Pressure Pressure test ( kg/cm 2 for 10 and 20 min) None Heat Heat provocation and threshold test (warm water) None Sunlight UV and visible light of a variety of wavelengths Dermographism Fric test CBC, ESR, CRP Vibration Vibration test None Aquagenic Wet cloth at body temperature for 20 min None Cholinergic Exercise and hotbath provocation None Contact Prick/patch test read after 20 min None NSAIDs, non-steroidal anti-inflammatory drugs; ACEIs, angiotensin-converting enzyme inhibitors; UV, ultraviolet rays; CBC, complete blood count; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein. Table 2. UAS UAS Number of wheals Intensity of pruritus 0 None None 1 < 20 per 24 hours Mild (present but not troublesome) per 24 hours Moderate (troublesome but does not interfere with normal daily activity or sleep) 3 > 50 per 24 hours Severe (interferes with normal daily activity or sleep) UAS over 7 days (UAS7): 28, severe; 6, mild UAS, urticaria activity score

5 - 예영민. 두드러기분류및진단 - 항히스타민제치료에대한반응여부등으로평가한다. 두드러기활성도점수는하루평균발생한팽진의갯수와가려움의심한정도를각각 0-3점까지측정하여두항목점수를합하여총 0-6점까지의점수로계산한다 (Table 2). 특히 1주일간의활성도를평가한 UAS7 (0-42) 이가이드라인에서권장되고있다 [1]. 최근개발된 UCT는지난 4주간의두드러기증상의조절정도를환자가평가하는 4문항으로구성된척도로총 16점중 12점이상인경우잘조절되는두드러기로평가할수있다 [1,5]. 두드러기는질병활성도뿐아니라환자의삶의질평가도매우중요하며이를측정하기위한평가척도가개발되었다 [1,6]. 혈관부종이동반된경우두드러기중증도가더높고삶의질저하도큰것으로나타나문진시이에대한확인도필요하다. 또한최근가이드라인은만성두드러기에서단계별약물치료를권장하고있고, 1차적으로졸리지않은 2세대항히스타민제를 4정또는 4종까지병용또는증량하는것을권고하고있어항히스타민제로잘조절되는환자와조절되지않는환자로임상적분류를하고있다. 약 50% 의환자는항히스타민제로조절되지않는두드러기로분류된다. 결론두드러기는유병기간에따라급성과만성으로분류하며, 급성두드러기의경우원인 ( 약물, 식품, 감염등 ) 을확인하여회피하도록해야한다. 최근국내외적으로만성두드러기의유병률이증가하고있다 [7,8]. 6주이상지속적으로발생또는예측할수없게악화되는팽진 / 혈관부종과가려움으로삶의질저하는심각하지만, 아직질병에대한사회적인식은낮은상태이다. 따라서원인확인, 조기진단및중증도평가를통한적절한유지치료에대한미충족수요가높다. 증례는 3개월전부터시작된만성자발성두드러기에진통소염제과민증과혈관부종이동반된환자로진단할수있다. 진통소염제복용으로인한급성악화시이에대한급성기치료도중요하지만, 원인약물에재노출되지않도록교육하고, 정보를다른의료진과공유하는것이필요하다. 만성두드러기는다양한원인에의하여발생할수있으며, 환자에따라악화인자도다양하다. 자가면역기전에의한만성자발성두드러기가 30-50% 를차지하며, 물리적자극에의해서유발되는만성유발성두드러기환자도있다. 알레르기질환및자가면역질환이일반인구에비하여흔히동반되며물리적자극, 가성알레르겐, 약물, 식품등이악화요인으로작용할수있어환자의임상양상에맞추어관련검사를진행하고, 확인된원인에대해서는철저한교육이필요하다. 중심단어 : 두드러기 ; 혈관부종 ; 급성두드러기 ; 만성두드러기 REFERENCES 1. Zuberbier T, Aberer W, Asero R, et al. The EAACI/GA 2 LEN/ EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria. Allergy 2018;73: Maurer M, Hawro T, Krause K, et al. Diagnosis and treatment of chronic inducible urticaria. Allergy 2019 May 18 [Epub] Sánchez J, Amaya E, Acevedo A, Celis A, Caraballo D, Cardona R. Prevalence of inducible urticaria in patients with chronic spontaneous urticaria: associated risk factors. J Allergy Clin Immunol Pract 2017;5: Ye YM, Jang GC, Choi SH, et al. KAAACI Work Group report on the management of chronic urticaria. Allergy Asthma Respir Dis 2015;3: Lee JH, Bae YJ, Lee SH, et al. Adaptation and validation of the Korean version of the urticaria control test and its correlation with salivary cortisone. Allergy Asthma Immunol Res 2019;11: Choi WS, Lim ES, Ban GY, et al. Disease-specific impairment of the quality of life in adult patients with chronic spontaneous urticaria. Korean J Intern Med 2018;33: Lee N, Lee JD, Lee HY, Kang DR, Ye YM. Epidemiology of chronic urticaria in Korea using the Korean health insurance database, Allergy Asthma Immunol Res 2017;9: Wertenteil S, Strunk A, Garg A. Prevalence estimates for chronic urticaria in the United States: a sex- and age-adjusted population analysis. J Am Acad Dermatol 2019;81:

The clinical experience of anti-IgE antibody treatment in patients with refractory chronic urticaria

$The clinical experience of anti-IgE antibody treatment in patients with refractory chronic urticaria$ The Clinical Experience of Omalizumab Treatment In Patients with Refractory Chronic Urticaria Young-Hee Nam, 1 Joo-Hee Kim, 2 Hyun Jung Jin, 3 Eui-Kyung Hwang, 4 Yoo-Seob Shin, 4 Young-Min Ye, 4 Hae-Sim