소아 청소년과 청주지회 최 용 재 소아과 전문의 / 다솜의원

AD with Herpes infection

아토피 피부염의 중증도 측정 AD Scoring systems 한 질환에 대한 연구와 진료--EBM 현대의학 - -분명한 정의와 진단을 요구함

Why do we score the AD? 수치로 정의되는 AD 치료효과를 산술적으로 표현 다기관 공동 연구의 도구- EBM 궁극적 목표 같은 parameter를 평가 관찰자간의 개인차를 최소화함

Diagnosis of Atopic Dermatitis Criteria by Hanifin and Rajka(1980) Criteria by UK working party(1994)

Hanifin and Rajka’s criteria for AD 1980

Typical morphology & distribution: Major Criteria (must have at least three) Pruritus Typical morphology & distribution: Adults: flexural lichenification or linearity Children & infants: facial and extensor surfaces Chronic or relapsing dermatitis Personal or family history of atopy

Pruritus Pruritus At one time, AD theory held that the itch came before the rash--it was often referred to as "the itch that rashes." Now there is evidence to suggest otherwise. The primary eruption is one characterized by dryness and erythema. Frequently parents of an infant with AD are aware of this "dryness" right from birth. However, itchiness becomes prominent early in the disease process as well.

Typical morphology & distribution Children Often the first skin changes are quite subtle, but the itching is usually evident. The first presentation of AD is one of mild patchy erythema and scaling over the infant's cheeks as well as the extensor surfaces of the legs. These lesions are poorly demarcated. As the disease progresses and spreads, large areas of the body become covered with dry, reddened skin. In General A favourite area for AD to present is behind the ears. Infra-auricular fissures are often seen in patients in whom the disease is more severe. The scalp may become dry and scaly as well, but this scaling is different from that seen in "cradle cap" (seborrheic dermatitis) in which the scale is large, yellow, and greasy. Adults The disease affects flexural areas primarily. The skin surface in these locations becomes chronically inflamed (reddened) and lichenified (thickened) after years of being rubbed. The thickening is easily recognized by the prominence of the skin lines as well as the "leathery" feel of the skin surface.

Adult AD distribution

Chronic or relapsing dermatitis

Personal or family history of AD

Minor Criteria (must have at least 3) Xerosis Icthyosis/keratosis pilaris/palmer hyperlinearity Immediate (type 1) skin test reactivity Elevated serum IgE Early age at onset 3. Type 1 skin test reactivity Atopics may demonstrate an increased tendency toward this type of reaction which follows exposure to an antigen to which they have been previously sensitized. In its simplest form, this reaction presents as a hive or "wheal" (urticaria), but it may also be manifest as angioedema, anaphylaxis, hay fever, or asthma. 4. Elevated serum IgE Patients with AD have a hereditary abnormality in the immune mechanism so that there is increased production of IgE antibodies. There appears to be a degree of positive correlation between the amount of IgE and the extent and severity of the AD.

Xerosis Xerosis This is a term applied to any condition of the skin in which dryness is more than the normal state. The clinical appearance is one of dryness with fine lines and scaling in a pattern over the skin. It may or may not be intermittently associated with erythematous patches, and excoriation is not a common feature. The condition often involves the legs.

Icthyosis/keratosis pilaris/palmer hyperlinearity Palmar hyperlinearity Frequently the hands of younger patients will appear "old-looking"--it is always a surprise to see a 1-year old with the hands of a 99-year old! This appearance is due to hyperlinearity. Kerotosis pilaris This is a common autosomal dominant trait, frequently associated with AD. There is follicular hyperkeratosis which is seen on the extensor surfaces of the arms and legs, and occasionally on the face as well. It gives the skin the appearance of permanent "goose bumps" and a rough texture. Ichthyosis vulgaris This is a disorder of keratinization, most often seen alone but it can be associated with AD. It is an autosomal dominant inherited disorder characterized by varying degrees of dryness, scaling, and exfoliation of skin. The scales found on the scalp are quite small, but increase in size in a caudal direction over the rest of the body. On the legs they are large and have a "pasted-on" appearance.

Minor Criteria (must have at least 3) Tendency to skin infections Hand/foot dermatitis Nipple eczema Conjunctivitis Dennie-Morgan fold

Tendency to skin infections S. aureus, herpes simplex impaired cellular immunity Tendancy to skin infections There is evidence to suggest that AD patients have a deficiency in cell-mediated immunity. One manifestation of this is that their skin often becomes secondarily infected with Staphlocccus aureus (S. aureus). When this occurs it has the appearance of golden crusts on top of the excoriated papules and plaques. This infection may be accompanied by marked lymphadenopathy. Another manifestation is the increased incidence of viral skin infection, for example with herpes simplex virus. When this occurs, the term "Kaposi's varicelliforme eruption" is used. The herpetic lesions are more widespread than in the usual case of cold sores--there may be hundreds to thousands of individual lesions. A common viral skin infection in children with AD is molluscum contagiosum. In AD patients these infections are characterized by many more umbilicated papules than are seen in similarly infected non-atopics. AD patients are also more susceptible to the development of warts (verrucae) and fungal skin infections.

Hand/foot dermatitis Hand/foot dermatitis This is a recurring or persistent localization of dermatitis to the wrists, palms, or fingers of the hand, or the soles of the feet. It can be exacerbated by contact with offending allergens, e.g., soaps, solvents, or metals for the hands, dyes for the feet. It is usually associated with atopy and xerosis. One form, called "winter feet," usually affects older children and has a clinical picture of painful, mildly erythematous, dry scaling and fissuring of the sole, particularly the toes and ball, of the foot.

Nipple eczema Nipple eczema When seen in AD patients, this is usually a chronic bilateral condition characterized by a moist patch of dermatitis localized to the nipple and areola area. It responds to glucocorticoid treatment. It is important because the appearance can mimic Paget's disease of the nipple, a serious condition which is usually unilateral and associated with a small but chronic discharge, and indicative of an underlying ductal carcinoma of the mammary gland. Paget's does not respond to corticosteroid therapy.

Conjunctivitis

Dennie-Morgan fold Dennie-Morgan fold This is a pleat of skin (line or groove) that appears in the lower eyelid during the first few months of life and may be related to edema of the lid. Although more a marker of atopy than of AD, it can be a clinically useful sign of AD in Caucasians. However, in black races these infraorbital folds are extremely common and bear no relationship to the development of AD. The folds remain throughout life, but become much less prominent in old age.

Minor Criteria (must have at least 3) Keratoconus Anterior subcapsular cataracts Orbital darkening Facial pallor/erythema Pityriasis alba Anterior neck folds

Keratoconus Keratoconus This is an elongation and protrusion of the corneal surface of the eye that occurs infrequently (1%) in AD patients and is independent of cataract formation. Frequent rubbing of the eyes may be the explanation for these degenerative changes that have their onset in childhood.

Anterior subcapsular cataracts AD patients have an increased incidence of developing bilateral, central, shield-shaped, asymptomatic cataracts that involve the anterior and posterior superficial cortex or both. In AD patients, these cataracts develop at an earlier age and mature more quickly than do senile cataracts.

Orbital darkening, central pallor, erythema Orbital darkening, central pallor, and erythema There is generalized pallor especially in the areas of the nose, mouth and ears. Erythema of the cheeks and darkening around the eyes is a typical facial pattern seen in atopic patients, particularly children. This typical appearance of the eye is referred to as an "atopic shiner" because of the greyish purple infraorbital discoloration, perhaps due in part to frequent rubbing of the eyes.

Pityriasis alba Pityriasis alba This is a self-limiting, recurrent, condition characterized by asymptomatic, hypopigmented, finely scaling patches often seen on the cheeks of children. These plaques can be found on outer parts of the upper arms and the thighs as well. Lesions are more obvious in the summer because they do not tan. This loss of pigmentation is not permanent.

Anterior neck fold

Minor Criteria (must have at least 3) Itch when sweating Intolerance to wool & lipid solvents Perifollicular accentuation Food intolerance Course influenced by environmental/emotional factors White demographic/delayed blanch

Itch when sweating Pruritus with perspiration Sweating induces intense itching and burning in the skin of AD patients. They will notice that their disease is worse on very hot, humid days. They may also report that the itching is worse after a bout of prolonged exercise or stress. Rapid changes in temperature are not well-tolerated. A rapid cooling of the skin will trigger an itch, for example removing clothes to get ready for bed.

Intolerance to wool & lipid solvents Intolerance to wool and lipid solvents This is generally more of an irritation and resulting inflammation rather than a contact allergic reaction. The irritation causes increased itching and the cycle begins.

Perifollicular accentuation Chronic rubbing of the skin will frequently produce tiny papules which on closer examination are seen to surround hair follicles.

Food intolerance

Course influenced by environmental/emotional factors Influence of environmental/emotional factors These factors appear to play a modifying role in the course of AD. The effects of emotions, extremes of temperature and humidity, as well as texture of clothing have all been documented with respect to AD patients. There is altered peripheral cutaneous vasoconstriction of affected skin in atopics with a decreased response to cooling and warmth and increased perspiration in the flexural regions.

White dermographic/delayed blanch White dermographism/delayed blanch This is a curious blanch phenomenon seen after stroking the skin in which the normal red line and flare of the non-atopic individual is quickly replaced with a white line and no flare. Another blanching reaction occurs when acetylcholine is injected following the development of the red flare. Both of these can be seen in eczematous skin affected with contact dermatitis, but are not diagnostic. There are also paradoxical vasomotor changes seen in response to acetylcholine, nicotinic acid esters, and histamine.

diagnostic criteria for AD UK Working Party diagnostic criteria for AD From Williams HC, et al. The UK Working Party’s diagnostic criteria for AD : Derivation of a minimum set of discriminators for AD. Br J Dermatol 1994;31:386 –96.

UK Working Party diagnostic criteria for AD 12개월간 지속된 가려움증 UK Working Party diagnostic criteria for AD

아래 항목중 3가지 이상 the skin creases such as antecubital, popliteal, dorsal ankles or neck asthma or hay fever 지난 한해동안 건조한 피부 Visible flexural dermatitis 2세 이전의 발병 • Hx of involvement of the skin creases such as antecubital, popliteal, dorsal ankles or neck • A hx of asthma or hay fever (or history in 1st degree relative in those under the age of 4) • A hx of generally dry skin during the last year • Visible flexural dermatitis (or dermatitis of checks or forehead and outer limbs in children less than the age of 4) • Onset under the age of 2 (not used if child is under the age of 4)

Rajka’s AD score Rajka Costa’s socre Costa and co-workers 1989 ADASI (Atopic Dermatitis Area and Severity Index) Bahner et al 1991 EASI NESS

Costa’s score-simpler one 10 signs and symptoms(0~7) = Most severely affected area 10 different skin areas(0~3) The maximum possible socre –100 70 from symptoms and signs 30 form extension

SCORAD 객관적 징후 severity Extention 주관적 징후 pruritus loss of sleep (Eurpean Task Force on Atopic Dermatitis, 1993) 객관적 징후 severity Extention 주관적 징후 pruritus loss of sleep

Reference evaluation system 1 Extent(0-100) Intensity(0-18) 6 items (0,1,2,3) Subjective symptoms (0-30) VAS(0-10) Pruritus Sleep loss Overall skin condition The last 3days/nights How much does skin condition interfere with everyday life?

SCORAD의 객관적 징후 홍반(Erythema) 부종/구진(Edema/papulation) 진무름/가피(Oozing/crusts) 찰상(Excoriation) 태선화(Lichenification) 건조증 Half point – not recommended Exclude one target area or worst area

What do we measure?

홍반(Erythema)0,1,2,3

Edema/papulation of SCORAD Palpable infiltration of the skin Difficult to define with photographs Palpation of the lesion Induration is discarded.- equivocal meaning

Edema/papulation of SCORAD

Oozing/Crusts of SCORAD Exudative lesions resulting from epidermal edema and vesiculation

Oozing/Crusts of SCORAD

Excoriations of SCORAD An objective marker for pruritus More visible in nonlichenified lesions

Excoriations of SCORAD

Lichenification of SCORAD Epidermal thickening in chronic lesions Grossly accentuated skin furrows separate shiny rhomboid area. The color is graysh or brownish Prurigo lesions and common large fold involvement ungdergo lichenification. In patients over 2years of age

Lichenification of SCORAD

Prurigo Nodularis

Dryness of SCORAD 염증부위와 떨어져 있는 부위 보습제를 사용하지 않은 부위 염증에서 회복되고 있는 부위는 제외 촉감 Icthyosis vulgaris in association Fissures–severe dryness on the xtremities Reference Evalutiaon system

침범 범위의 Grading The rule of nine with a age variant. Only inflammatoy lesions Not dryness One patent’s palm = 1% of his BSA

SCORAD http://adserver.sante.univ-nantes.fr SCORACARD http:/ww.tpsproduction.com by Pelosi and Tripodi The best validated systems Suited for clinical trials Too complicated Too time consuming ->TIS In addition to analogue parameters of Costa’s socre(A and B), subjective symptoms© are alos considered and calculated in the final score

SCORAD signs + symptoms Toward pediatric populations

EASI (the eczema area and severity index) Hanifin JM 2001

EASI (Eczema Area and Severity Index) Body region EASI Score Head/Neck (H) (E+I+Ex+L) x Area x 0.1(0.2<8세) Upper limb (UL) (E+I+Ex+L) x Area x 0.2 Trunk (T) (E+I+Ex+L) x Area x 0.3 Lower limbs (LL) (E+I+Ex+L) x Area x0.4(0.3< 8세) EASI = Sum of the above four body region scores. Area 0 = no eruption; 1= < 10%; 2 = < 10-29%; 3 = < 30 to 49%; 4 = < 50 to 69%;5 = < 70 to 89%;6 = > 90 ~100%. E=erythema, I=induration/papulation, Ex=excoriation, L = lichenification.

EASI PASI Different sign severity parameters Disease extent

EASI 각 부위를 Adaptable to children acute and chronic signs 개별적으로 평가한 후 합쳐서 산정 Adaptable to children acute and chronic signs Avoid mixing objective parameters with subjective signs Symptoms of pruritus and sleep loss (-)

EASI 2 non-negligible limits (the eczema area and severity index) Hanifin JM, 2001 2 non-negligible limits Not applicable to children < 2 years of age Ignores the cardinal symptoms (pruritus and loss of sleep) - m-EASI

SA-EASI(Self-Administered- EASI) Housman 2002 For large-scale epidemiological studies For A caregiver’s SA-EASI

SA-EASI VAS 항목 Redness Thickness Dryness Scratches Itchness

SASSAD (Six Area, Six Sign Atopic Dermatitis Severity Score) Bertha-Jones 1996

SASSAD A less sophisticated system A scale (0,1,2,3) ; maximum 108 Very rapid to use Pruritus or loss of sleep ; not included Significant interobserver variation 항목 1.Ertyhema 2.exudation 3.excoriation 4.dryness 5.cracking 6.lichenification 범위 1.Arms 2. hands 3.legs 4.feet 5.head and neck 6.trunk SASSAD (2min for an expert, 10 min for a novice)

SASSAD Wide interobserver variation Lichen. and dryness – most difficult Exud. and cracking – best agreement Erythema – poor to fair agreement Interobserver variation (C. R. Charman) SASSAD>SCORAD>EASI

TIS(three item severity score) Wolker-storfer A 1999 1.Erythema, 2.edema/papulation, 3 excoriation A scale (0,1,2,3) For routine clinical use For screening purposes

TIS and Scorad TIS SCORAD Mild 0-2 25이하 Moderate 3-5 26-55 Severe 6-9 56이상

(The objective Severity Assessment of Atopic Dermatitis score) OSAAD score Jeffrey Sugarman 2003 (The objective Severity Assessment of Atopic Dermatitis score) The computer-aided method of estimating BSA SC(stratum corneum) function Permeability barrier function (TEWL) SC hydration(CM(corneometry)) ::: OSAAD score = BSA x (TEWL+CM)

Scoring systems 전세계적인 표준이 없음. scoring systems간에 일치된 결과가 없음 IGA – subjectiv clinical scores SCORAD variability between expert & non expert ; they say, ”negligible” Benefit for clinicians Lab<clinical score

ICCAD II guidelines

Proposed treatment signal

HYPERSCORAD www.atopycare.net

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환자메뉴얼 모의테스트(step 1) / 침범영역 선택

모의테스트(step 1) / 대표부위에 대한 증상별 진행강도선택 환자메뉴얼 모의테스트(step 1) / 대표부위에 대한 증상별 진행강도선택

모의테스트(step 1) / 여섯가지 부위에 대한 증상별 진행강도선택 환자메뉴얼 모의테스트(step 1) / 여섯가지 부위에 대한 증상별 진행강도선택

모의테스트(step 2) / 여섯가지 부위에 대한 증상별 진행강도선택 환자메뉴얼 모의테스트(step 2) / 여섯가지 부위에 대한 증상별 진행강도선택

모의테스트(step 1) / 의약제와 자각증상에 대한 설문 환자메뉴얼 모의테스트(step 1) / 의약제와 자각증상에 대한 설문

모의테스트[step2 ( SCORAD )]) / 침범영역체크 환자메뉴얼 모의테스트[step2 ( SCORAD )]) / 침범영역체크

모의테스트[step2 ( SCORAD )] / 대표부위에 대한 증상별 진행강도선택 환자메뉴얼 모의테스트[step2 ( SCORAD )] / 대표부위에 대한 증상별 진행강도선택

모의테스트[step3( EASI & SASSAD) ]/ 이전단계에서 선택한 침범영역 확인 환자메뉴얼 모의테스트[step3( EASI & SASSAD) ]/ 이전단계에서 선택한 침범영역 확인

모의테스트[step3( EASI & SASSAD) ]/ 이전단계에서 선택한 침범영역 확인 환자메뉴얼 모의테스트[step3( EASI & SASSAD) ]/ 이전단계에서 선택한 침범영역 확인

환자메뉴얼 자가진단[step3 (EASI & SASSAD)] / 여섯가지 부위에 대한 증상별 강도선택

환자메뉴얼 자가진단[step3 (부증상에 대한 설문) ] / 처음 자가진단시 1회만 시행된다

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진료기록조회 / 환자자가진단기록 및 진료기록 리스트 환자메뉴얼 진료기록조회 / 환자자가진단기록 및 진료기록 리스트

진료기록조회 / 환자자가진단기록 및 진료기록 상세보기. 환자메뉴얼 진료기록조회 / 환자자가진단기록 및 진료기록 상세보기.

환자메뉴얼 진료기록조회 / 호전도 및 약물호전도

환자메뉴얼 진료하기 / 진료환자선택

진료하기 / 의용제 및 자각증상설문, 침범영역선택 환자메뉴얼 진료하기 / 의용제 및 자각증상설문, 침범영역선택

진료하기 / 대표부위에 대한 증상별 진행강도선택 환자메뉴얼 진료하기 / 대표부위에 대한 증상별 진행강도선택

진료하기 / 여섯가지 부위에 대한 증상별 강도선택 환자메뉴얼 진료하기 / 여섯가지 부위에 대한 증상별 강도선택

환자메뉴얼 모의테스트 결과 확인

AD with Herpes

AD with Herpes

AD with Herpes