Diagnosis and Japanese Guideline


Definition

Atopic dermatitis is a pruritic, eczematous dermatitis; its symptoms chronically fluctuate with remissions and relapses. Most individuals with atopic dermatitis have atopic diathesis

Atopic diathesis: (1) personal or family history (asthma, allergic rhinitis and/or conjunctivitis, and atopic dermatitis) and/or (2) predisposition to overproduction of immunoglobulin E (IgE) antibodies

Diagnostic criteria for atopic dermatitis

1. Pruritus

2. Typical morphology and distribution:

 (1) Eczematous dermatitis

Acute lesions: erythema, exudation, papules, vesiculopapules, scales, and crusts

Chronic lesions: infiltrated erythema, lichenification, prurigo, scales, and crusts

 (2) Distribution

Symmetrical

   Predilection sites: forehead, periorbital area, perioral area, lips, periauricular area, neck, joint areas of limbs, and trunk

Age-related characteristics

   Infantile phase: starts on the scalp and face, often spreads to the trunk and extremities

   Childhood phase: neck and the flexural surfaces of the arms and legs

   Adolescent and adult phase: tendency to be severe on the upper half of the body (face, neck, anterior chest, and back)

3. Chronic or chronically relapsing course (usually coexistence of old and new lesions):

More than 2 months in infancy

More than 6 months in childhood, adolescence, and adulthood

Definitive diagnosis of atopic dermatitis requires the presence of all three features without any consideration of severity. Other cases should be evaluated on the basis of the age and clinical course with the tentative diagnosis of acute or chronic, nonspecific eczema

Differential diagnosis (association may occur)

Contact dermatitis, seborrheic dermatitis, prurigo simplex, scabies, miliaria, ichthyosis, xerotic eczema, hand dermatitis (non-atopic), cutaneous lymphoma, psoriasis, immunodeficiency diseases, collagen diseases (SLE, dermatomyositis), and Netherton’s syndrome

Diagnostic aids

Family history (bronchial asthma, allergic rhinitis and/or conjunctivitis, atopic dermatitis), personal history (bronchial asthma, allergic rhinitis, and/or conjunctivitis), follicular papules (gooseskin), elevated serum IgE level

Clinical types (not applicable to the infantile phase)

Flexural surface type; extensor surface type; dry form in childhood; head, face, neck, upper chest, and back type; prurigo type; erythroderma type; combinations of various types are common

Significant complications

Ocular complication (cataract and/or retinal detachment), especially in patients with severe facial lesions; Kaposi’s varicelliform eruption; molluscum contagiosum; and impetigo contagiosa


Cited from ref. [2]



Eruption is symmetrically distributed and frequently develops on the forehead, periorbital area, perioral area, lips, periauricular area, neck, joint areas of limbs, and trunk. Its distribution is characterized by age. During infancy, eruption initially appears in the scalp and face, often expanding to the trunk and limbs. It appears in AD-specific sites during childhood, such as the neck and the flexural surfaces of the arms and legs. During adolescence/adulthood, it becomes marked in the upper body, including the face. AD-suspected patients are regarded as having acute or chronic eczema, and diagnoses are made based on their age and courses.

In a revision published in 2009, cutaneous lymphoma, psoriasis, immunodeficiency diseases (such as hyper-immunoglobulin E (IgE) syndrome and Wiskott-Aldrich syndrome), collagen disease (systemic lupus erythematosus and dermatomyositis), and Netherton’s syndrome were newly added as disorders to be ruled out (Table 20.1) [2]. Therefore, it is essential to differentiate these disorders and be familiar with the complications of AD.



20.1.1.2 Diagnostic Criteria by Hanifin and Rajka


The diagnostic criteria defined by Hanifin and Rajka in 1980 are frequently used worldwide [3]. One of the differences between Hanifin and Rajka’s criteria and the JDA criteria is that a personal or family history of atopic diseases (asthma, allergic rhinitis/conjunctivitis, and AD) is defined as a basic feature in the former and as a diagnostic aid in the latter. However, atopic diathesis is clearly addressed in the definition of AD by the JDA (Table 20.1).

The 23 minor features listed in the Hanifin and Rajka’s criteria are characteristic for AD and often observed in this disease. However, their frequencies of development vary, and discrete expressions are used for some of the features; therefore they are excluded from the diagnostic criteria by the JDA, although some of them are referred to as diagnostic aids, clinical types, or significant complications (Table 20.1). Subsequently, an “abridged edition of Hanifin and Rajka’s diagnostic criteria” was published in 2003 [4].


20.1.1.3 Nationwide Investigation by Inquiry/Questionnaire


A questionnaire for AD diagnosis was developed in 1994 by the UK Working Party, and it has been used worldwide [5]. Its Japanese translation version has proven to be useful [6]. Furthermore, based on the questionnaire developed by the International Study of Asthma and Allergies in Childhood (ISAAC), global epidemiological surveys about eczema, including AD, have been conducted periodically (http://​isaac.​auckland.​ac.​nz/​Index.​html) [7]. Its Japanese translation version is used for epidemiological surveys [8].



20.1.2 Severity Classification



20.1.2.1 Severity Classification for the Whole Body


The classification of the severity of AD prepared by the Atopic Dermatitis Severity Classification-Reviewing Committee of the JDA is available for clinical studies because its statistical reliability and usefulness have been verified (Fig. 20.1) [2, 9]. For the severity classification, three elements of eruption (erythema/acute papules, exudation/crusts, and chronic papules/nodules/lichenification) are evaluated in the most severely affected part of each of the five body sites (head/neck, anterior trunk, posterior trunk, upper limbs, and lower limbs). The areas of eruption on the five body sites are also evaluated, and both scores are totalized (Fig. 20.1) (maximum score, 60). As a simple method reviewed by this committee, a method to divide the whole body into the same five sites and calculate the sum of the global assessment scores of these sites is also presented (Fig. 20.2) (maximum score, 20) [2, 10].

A430132_1_En_20_Fig1_HTML.gif


Fig. 20.1
Severity classification of atopic dermatitis by the Japanese Dermatological Association (Cited from ref. [2])


A430132_1_En_20_Fig2_HTML.jpg


Fig. 20.2
Severity classification of atopic dermatitis by the Japanese Dermatological Association (simple method) (Cited from ref. [2])

In addition, as another simple method, the severity index has also been proposed by the Research Group of the Ministry of Health, Labour and Welfare (Table 20.2) [11]. Internationally, the Severity Scoring of Atopic Dermatitis (SCORAD) (maximum score, 103) [12] established by the European Task Force on Atopic Dermatitis or the Eczema Area and Severity Index (EASI) (maximum score, 72) [13] in the USA is widely used.


Table 20.2
Severity index











There are several criteria proposed for severity assessment of atopic dermatitis at present that require proficiency in assessment. Accordingly, the following severity levels are defined as indices for treatment.

Mild: Only mild rashes are observed irrespective of the area

Moderate: Rashes with severe inflammation are observed in less than 10% of the body surface area

Severe: Rashes with severe inflammation are observed ≥10% to <30% of the body surface area

Most severe: Rashes with severe inflammation are observed ≥30% of the body surface area

Mild rash: Lesions are seen chiefly with mild erythema, dry skin, or desquamation

Rashes with severe inflammation: Lesion with erythema, papule, erosion, infiltration, lichenification, etc.


Cited from ref. [11]


20.1.2.2 Severity Classification Considering Clinical Course


As a severity classification considering the clinical course, the system of grading of the severity of AD published by Rajka and Langeland is frequently used [14]. Additionally, in the abovementioned ISAAC questionnaire, the question “In the last 12 months, how often, on average, have you (has your child) been kept awake at night by this itchy rash?” diagnoses patients as severe when they answer “1 or more nights per week” [7].


20.1.2.3 Evaluation of Pruritus


The visual analogue scale (VAS) is useful for evaluating pruritus [15]. In this scale, 1 point is marked on a 10-cm axis in accordance with the degree of pruritus, and the distance (mm) from the left end to the marked point is evaluated as the pruritus scale score, regarding the left end “no itch” as zero and the right end “the worst imaginable itch” as 100. As described in SCORAD (evaluated by the scale of 0–10 in SCORAD), VAS can also be used for sleep loss.


20.1.2.4 Evaluation of Quality of Life (QOL)


The Skindex-16 and Dermatology Life Quality Index (DLQI) have been statistically analyzed [16, 17]. Their Japanese versions were published and have been applied to the treatment of various skin diseases including AD [18, 19].


20.1.2.5 Severity of Eruption


The primary treatment, application of topical corticosteroid, should be determined based on “the severity of each eruption” (Tables 20.3 and 20.4) [20]. Briefly, potent topical therapy is selected to treat severe eruption even when its extent is narrow. However, it is not required for patients with mild eruption even when its area is extensive. Therefore, “the severity of each eruption” is the most important factor to consider when selecting topical therapy, and a severity assessment must be performed by physicians with dermatological skills in order to evaluate severity and predict the treatment response.


Table 20.3
Severity of eruption and topical corticosteroid application




























Severity

Eruption

Topical corticosteroid application

Severe

Primarily severe swelling/edema/infiltration or erythema with lichenification, multiple papules, severe scales, crusts, vesicles, erosion, multiple excoriations, and pruriginous nodules

The use of very strong or strong-class topical corticosteroids is the first-line treatment

Strongest-class topical corticosteroids are also available for refractory pruriginous nodules if sufficient effects are not achieved by applying very strong-class topical corticosteroids

Moderate

Primarily moderate erythema, scales, a few papules, and excoriations

The use of strong- or medium-class topical corticosteroids is the first-line treatment

Mild

Primarily dryness, mild erythema, and scales

The use of medium- or weak-class topical corticosteroids is the first-line treatment

Slight

Primarily dryness with negligible inflammation

Topical application of medicines other than corticosteroids (emollients)


Cited from ref. [20]



Table 20.4
Rank of topical corticosteroids






















































Strongest

0.05%

Clobetasol propionate

0.05%

Diflorasone diacetate

Very strong

0.1%

Mometasone furoate

0.05%

Betamethasone butyrate propionate

0.05%

Fluocinonide

0.064%

Betamethasone dipropionate

0.05%

Difluprednate

0.1%

Amcinonide

0.1%

Diflucortolone valerate

0.1%

Hydrocortisone butyrate propionate

Strong

0.3%

Deprodone propionate

0.1%

Dexamethasone propionate

0.12%

Dexamethasone valerate

0.1%

Halcinonide

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Apr 13, 2018 | Posted by in Dermatology | Comments Off on Diagnosis and Japanese Guideline

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