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Scoring systems in dermatology

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1 Scoring systems in dermatology

2 How does your knowledge of scoring systems in dermatology stack up ?

3 Scoring systems in dermatology IJDVL 72L315 n 2OO6
Scores are used to evaluate the severity of skin diseases. This is particularly important for monitoring the response to therapy and for evaluating the efficacy of new drugs. This review deals with scores that are commonly used for common skin diseases & assessing the severity of dermatological disease in clinical research.

4 ATOPIC DERMATITIS Scores that are commonly used for objective assessment of atopic dermatitis are 1. SCORing Atopic Dermatitis (SCORAD) 2. The Six Area, Six Sign Atopic Dermatitis (SASSAD) severity score IJDVL 72: 315, 2OO6 1. SCORAD (SCORing Atopic Dermatitis) Developed by the European Task Force on atopic dermatitis in 1993, it is the most commonly used scoring system for measuring the severity of atopic dermatitis. It is used to standardize the assessment of atopic dermatitis and to help in the interpretation of therapeutic studies. The SCORAD Index is a composite score based on 3 subscores: A = The extent score based on body surface area calculated using the 'Rule of 9'. B = Intensity score based on 6 clinical findings in atopic dermatitis, namely erythema, edema or papulations, oozing or crusting, excoriation, lichenification, dryness, graded on a scale of (0- absent, 1- mild, 2- moderate, 3- severe). C = The score for pruritus and sleep loss graded on a visual analog scale of 0 to 10. The severity is based on the average extent for the last 3 days or nights. Final formula for calculation of SCORAD is as follows: SCORAD = A/5 + 7(B/2) + C The disadvantage of this scoring system is the significant interobserver variation which makes subsequent assessment of the patient by the same observer necessary. 2. SASSAD The Six Area, Six Sign Atopic Dermatitis severity score has proved to be a simple and effective system for recording and monitoring disease activity in atopic dermatitis. The score is obtained by grading six signs (erythema, exudation, excoriation, dryness, cracking and lichenification), each on a scale of 0 (absent), 1 (mild), 2 (moderate), or 3 (severe), at each of six sites (arms, hands, legs, feet, head and neck, trunk). The maximum score is 108. A modified version of the SASSAD known as the six-area 'total body severity assessment (TBSA)'has also been described. The TBSA, which has a maximum score of 108, differs from SASSAD in that it assesses infiltration and vesicles and/or papules, and excludes lichenification. Other scores in AD that are uncommonly used are listed in the following table.

5 Scores in atopic dermatitis

6 SCORAD SCORAD = SCORing Atopic Dermatitis, cinical tool for assessing the severity of atopic dermatitis as objectively as possible A) Extent criteria - determine the approximate percentage of body surface covered by eczema, using the rule of 9s (e.g. 50% involvement of right lower limb = 9% of body surface area)

7 SCORAD (Continue) B) Intensity criteria - score the features below on a scale of 0-3. Choose a lesion that is representative of most of the patient's eczema (not the worst or the best-looking lesion). Score xerosis on an area of skin without eczema/inflammation. Scale 0 - None 1 - Mild 2 - Moderate 3 - Severe Features Erythema Edema/papulation Oozing/crusting Excoriation (scratch marks) Xerosis (dryness) Lichenification (thickening) C) Subjective symptoms - score the features below on a visual analogue scale of 0-10 (0 - none, 10 - worst imaginable) Pruritus Insomnia (sleep disturbance) Final score = A/5 + 7B/2 + C 0 - Lowest possible score, Highest possible score Interpretation <25 - Mild AD 25–50 - Moderate AD >50 - Severe AD

8 Investigator's Global Assessment (IGA) The Investigator graded the global assessment of each subject's atopic area using the following scale

9 Atopic Dermatitis Severity Index (ADSI) The summary score from the 5 clinical features of atopic dermatitis - erythema, pruritus, exudation, excoriation, and lichenification - was assessed on each subject's selected target lesion using the following 4-point scales

10 Ideal tubs for babies between 6 to 24 months

11

12 TRADITIONAL ASSESSMENT TOOLS of psoriasis
The PASI ( psoriasis area severity index) is a measure of the average redness, thickness, and scaliness of the lesions (each graded on a 0–4 scale), weighted by the area of involvement . Another key measure used in clinical trials of psoriasis is the physician global assessment (PGA). Global assessments can be done for extensive disease as well as localized plaques. When conducting a clinical trial for treatment for psoriasis, a predetermined primary endpoint is required on which the efficacy of the drug will be assessed. Quality of life measures are helpful for demonstrating that changes in the severity of the skin lesions correspond to improvement in patients’ lives.

13 Other scores in psoriasis

14 Psoriasis area severity index (PASI)
  Psoriasis area severity index (PASI) This is currently the gold standard score for the assessment of extensive psoriasis, but has the limitation of interobserver variation. Four sites of affection, the head (h), upper limb (u), trunk (t) and lower limbs (l), are separately scored by using three parameters, erythema, induration and desquamation, each of which is graded on a severity scale of 0 to 4, where 0 = nil, 1 = mild, 2 = moderate, 3 = severe and 4 = very severe. The area-wise percentage involvement of the involved sites is calculated as: 1 = less than 10% area; 2 = 10-29%; 3 = 30-49%; 4 = 50-69%; 5 = 70-89%; and 6 = more than 90%. The final formula for PASI score is: PASI = 0.1 (Eh + Ih + Dh) Ah (Eu + Iu + Du) Au (Et + It+ Dt) At (El + Il + Dl) Al The maximum score of PASI is 72. PASI 75 is a 75% reduction of baseline PASI score. It is commonly considered as a denominator for satisfactory results of any treatment modality for psoriasis. Other scores that are used for psoriasis are stated in the following table.

15 Psoriasis assessment tools in clinical trials Ann Rheum Dis 64: 65, 2OO5
*Steps in generating PASI score (a) Divide body into four areas: head, arms, trunk to groin, and legs to top of buttocks. (b) Generate an average score for the erythema, thickness, and scale for each of the 4 areas (0 = clear; 1–4 = increasing severity)†. (c) Sum scores of erythema, thickness, and scale for each area. (d) Generate a percentage for skin covered with psoriasis for each area and convert that to a 0–6 scale (0 = 0%; 1 = <10%; 2 = 10–<30%; 3 = 30–<50%; 4 = 50–<70%; 5 = 70–<90%; 6 = 90–100%). (e) Multiply score of item (c) above times item (d) above for each area and multiply that by 0.1, 0.2, 0.3, and 0.4 for head, arms, trunk, and legs, respectively. (f) Add these scores to get the PASI score. †Erythema, induration and scale are measured on a 0–4 scale (none, slight, mild, moderate, severe) ‡Area scoring criteria (score: % involvement) 0: 0 (clear) 1: <10% 2: 10–<30% 3: 30–<50% 4: 50–<70% 5: 70–<90% 6: 90–<100%

16 Example of PASI score

17 PASI calculator

18 Psoriatic BSA represents the percentage of total body surface (BSA) affected by psoriasis
The body has been divided into four segments, which are listed in column one of the above table and their approximate percentage of the entire body area is in column two. These approximate percentages are used as references and they represent the maximum that can be reported in the sixth column. The areas of the body segments affected (columns three and five) are to be determined using the "rule of palm" ("rule of palm" reference is based on the size of the patient's palm and not the assessor's palm). The percentages of anterior and posterior sites affected by psoriasis are to be determined as percentages of the total BSA. The final row and column are to be used to sum the totals of the four body segments and to calculate the total BSA

19 "Physician's Global Assessment (PGA) PGA represents the evaluation by the physicians of the global response of all psoriatic lesions to therapy.

20 Psoriasis quality-of-life questionnaire-12 (PQOL-12 score)

21

22

23 Severity of alopecia tool score (SALT score)
Dyshidrotic eczema area and severity index (DASI) Based on the severity grade of single items - number of vesicles per square centimetre (V), erythema (E), desquamation (S) and itch (I) - and the extension of the affected area (A) and is calculated with defined score points (p) as: DASI = (pV + pE = pS + pI) ´ pA. DASI was found to be a simple and useful tool to assess the severity of dyshidrotic eczema and the effect of therapy. Thus application of mind helps to design scores for semi-objective assessment of skin diseases. Till better objective parameters are developed, scores will continue to remain the gold standard for assessing the severity of dermatological diseases in clinical research. Severity of alopecia tool score (SALT score) In a study comparing efficacy of azelaic acid and anthralin for patchy alopecia areata, Sansaz et al used terminal hair regrowth score (RGS) which encompasses a scale ranging from 0 (inadequate response) to 2 (complete response). National Alopecia Areata Foundation working committee has devised "Severity of Alopecia Tool score" (SALT score). Scalp is divided into 4 areas namely, Vertex - 40% (0.4) of scalp surface area; right profile of scalp - 18% (0.18) of scalp surface area; left profile of scalp - 18% (0.18) of scalp surface area; Posterior aspect of scalp - 24% (0.24) of scalp surface area. Percentage of hair loss in any of these areas is percentage hair loss multiplied by percent surface area of the scalp in that area. SALT score is the sum of percentage of hair loss in all above mentioned areas. For e.g., if the percentage hair loss in vertex, right profile, left profile and posterior aspect is 20, 30, 40 and 50% respecively; then, SALT score = (20 ´ 0.4) = (30 ´ 0.18) + (40 ´ 0.18) + (50 ´ 0.24) = = 32.6

24 The effects of BOTOX® which may spread from the area of injection to produce symptoms consistent with botulinum toxin effects. These may include asthenia, generalized muscle weakness, diplopia, ptosis, dysphagia, dysphonia, dysarthria, urinary incontinence, and breathing difficulties. These symptoms have been reported hours to weeks after injection. Swallowing and breathing difficulties can be life threatening, and there have been reports of death Botox (cosmetic purposes) used to treat axillary hyperhidrosis Symptoms occur 1-2 days after exposure

25 SCORTEN scale The SCORTEN scale (SCORe of Toxic Epidermal Necrosis) is a severity-of-illness scale with which the severity of certain bullous conditions can be systematically determined. It was originally developed for toxic epidermal necrolysis, but can be used with burn victims, sufferers of Steven-Johnson syndrome, cutaneous drug reactions, or exfoliative wounds. These conditions have in common that they compromise the integrity of the skin and/or mucous membranes. In the SCORTEN Scale 7 independent risk factors for high mortality are systematically scored, so as to determine the mortality rate for that particular patient. Risk factor Age < 40 years > 40 years Associated malignancy no yes Heart rate (beats/min) < >120 Serum BUN (mg/dL) < >27 Detached or compromised body surface <10% >10% Serum bicarbonate (mEq/L) > <20 Serum glucose (mg/dL) < >250 The more risk factors present, the higher the SCORTEN score, and the higher the mortality rate, as shown in the following table. No of risk factors Mortality rate % % % % 5 or more >90%

26 The SCORTEN clinical scoring system for predicting outcome in TEN
TOXIC EPIDERMAL NECROSIS (TEN)  The score commonly used for assessing the patients of TEN is SCORTEN.  Scoring is based on the evaluation of seven independent risk factors within the first 24 hours of admission. One point is assigned to each variable. The value of the total number of points determines the predicted mortality: 0-1 points-3.2% mortality; 2 points-12.1%; %; %; ³5-90%.  

27 Adverse cutaneous reactions to drugs J. Postgraduate M. 42: 15, 1996
 Evaluation of adverse drug reactions; a stepwise approach  First Step: History and Physical Examination: * Temporal correlation with the drug * Incubation period (interval between the introduction of the drug and the onset of reaction) * Clinical pattern of the eruption * Any improvement on drug withdrawal * Any reaction on re-administration of the drug Second Step: In vivo Testing i) Rechallenge (Test Dosing): A positive rechallenge is generally accepted as strong evidence for causality. Reexposure should not be performed after a serious reaction. If a reaction fags to recur on rechallenge. it may be due to the fact that the previous reaction was a result of drug interaction. ii) Patch Testing: It is a well-known method of rechallenging the skin in conditions when cell mediated immunity is suspected viz-maculopapular eruptions, FDE, exfoliative dermatitis and lichenoid dermatitis. iii) Dechallenge: Most adverse reactions to drugs should remit with dechallenge i.e. withdrawal of the drug]. Third Step: In Vitro Testing Immunoassays such as radioallergosorbent lest (RAST) and Enzyme linked Immunosorbent Assays (ELISA) are in varying stages of development for the detection of IgE to aminoglycosides, penicillin, sulfonamides, ACTH etc. Other tests include Lymphocyte Transformation Test (LTT), Macrophage Migration Inhibition Factor Test (MIF), Lymphocyte Toxicity Assay (LTA), Basophil Degranulation Test, Histamine Release Test, Haemagglutination Assays 108 and flowcytometry. But unfortunately most in vitro tests 3 are unreliable for routine clinical use and are suitable only for immunologic research.

28 Colichicine is highly toxic
(Colchicum autumnale

29      Vitiligo  Two scores designed for the assessment of vitiligo are vitiligo area severity index (VASI)  and vitiligo disease activity score (VIDA). Vitiligo area severity index (VASI) The percentage of vitiligo involvement is calculated in terms of hand units. One hand unit (which encompasses the palm plus the volar surface of all digits) is approximately equivalent to 1% of the total body surface area. The degree of pigmentation is estimated to the nearest of one of the following percentages: 100% - complete depigmentation, no pigment is present; 90% - specks of pigment present; 75% - depigmented area exceeds the pigmented area; 50% - pigmented and depigmented areas are equal; 25% - pigmented area exceeds depigmented area; and 10% - only specks of depigmentation present. The VASI for each body region is determined by the product of the area of vitiligo in hand units and the extent of depigmentation within each hand unit measured patch. Total body VASI = S All body sites [Hand Units] ´ [Residual depigmentation] Vitiligo disease activity score (VIDA) The VIDA is a six-point scale for assessing vitiligo activity. Scoring is based on the individual's own opinion of the present disease activity over time. Active vitiligo involves either expansion of existing lesions or appearance of new lesions. Grading is as follows: VIDA Score +4 - Activity of 6 weeks or less duration; +3 - Activity of 6 weeks to 3 months; +2 - Activity of months;+1 - Activity of months; 0 - Stable for 1 year or more; and -1 - Stable with spontaneous repigmentation since 1 year or more. A low VIDA score indicates less activity.    

30 Vitiligo Area Scoring Index (VASI)
To calculate the total pigment loss you will need to know two things: The body surface area (BSA) affected. The actual percentage of pigment loss of each area. 1.  Determine the BSA affected. By dividing your skin surface into the following six separate regions: hands upper extremities   trunk face/neck lower extremities (which includes the groin and buttocks) feet 2. Determine how much BSA is involved with vitiligo for each specific region. The affected areas in each region are measured in hand units (a hand unit is defined as the size of the palm and is equal to 1% of the body surface). For example, if the surface area of involved skin is equivalent to five hand units, 5% of the body is considered involved with vitiligo. 3. Determine the percentage of depigmentation for each area. The percentage of depigmentation is calculated to the nearest one of the following percentages: 0, 10, 25, 50, 75, 90 or 100%.     1.00 (100%) - complete depigmentation, no pigment is present; .90 (90%) - specks of pigment present .75 (75%) - depigmented area exceeds the pigmented area; .50 (50%) - pigmented and depigmented areas are equal .25 (25%) - pigmented area exceeds depigmented area; .10  (10%) - only specks of depigmentation present.   4.  Determine the total depigmentation for the whole body. To determine the total depigmentation percentage for the entire body, you add the totals for each region. Working example of VASI formula: If the Upper Extremities region has 2 hand units (2%) of BSA affected, and is .75 (75%) depigmented, then the total depigmentation for the Upper Extremities is 1.5% of the body.  (.75 x 2 = 1.5%) If the Face/Neck region has 5 hand units (5%) of BSA affected, and the depigmented areas are 1.00 (100%) depigmented, then the total depigmentation for the Face/Neck is 5% of the body.  (5 x 1 = 5%) The total depigmentation for the entire body is found by adding the totals together. In the example above, the total depigmentation is 6.5%   (1.5% + 5% = 6.5%)      

31 Standardized assessments for estimating the degree of pigmentation to derive the Vitiligo Area Scoring Index . At 100% depigmentation, no pigment is present; at 90%, specks of pigment are present; at 75%, the depigmented area exceeds the pigmented area; at 50%, the depigmented and pigmented areas are equal; at 25%, the pigmented area exceeds the depigmented area; and at 10%, only specks of depigmentation are present. Wood’s lamp examination

32 Vitiligo disease activity (VIDA) score
The definition of active or stable vitiligo is not the same in different clinicians' viewpoints. To date, disease activity is mainly assessed based on medical history and physical examination. Thus, the size of lesions along with their number and the grade of repigmentation are recorded using photographic tools in any follow-up visit . One of the vitiligo-associated skin manifestations is Kӧbner phenomenon, defined as " the development of vitiligo lesions at sites of specifically traumatized skin". Some epidemiological studies have shown that this phenomenon occurs in most patients with vitiligo . Its clinical relevance is not established; however it seems that Kӧbner may indicate disease activity . . Disease activity VIDA Score Active, in the past 6 weeks Active, in the past 3 months Active, in the past 6 months Active, in the past 1 year Stable, for at least 1 year O Stable, for at least 1 year and spontaneous repigmenting *Active refers to expansion of existing lesions or appearance of new lesions; stable refers to condition when these symptoms are not present. Vitiligo disease activity (VIDA) score based on the patient's own opinion . Assessment of different vitiligo treatment options is difficult because there is no standardized scoring system for vitiligo . The percentage of depigmentation in association with total body surface can be estimated by using the hand-palm rule ,i.e., a lesion in the size of the patient's palm is equal to 1% of the total body surface . Vitiligo Area Scoring Index or VASI can be achieved by this rule. For each body region, the VASI is determined by the hand unite rule. The total body VASI is calculated by the following formula by considering the contribution of all body regions (possible rang: 0-100) : VASI= ∑ [Hand Unites] X [Residual Depigmentation] Because VASI is not accurate, Van Geel and colleagues have introduced a more accurate manner so-called a digital image analysis system for assessing vitiligo lesion surfaces both before and after different therapeutic modalities). Patients with vitiligo have a high prevalence of autoimmune thyroid disease or other autoimmune diseases; so, thyroid function should be checked in these patients .

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34 Scleroderma  Scores used for scleroderma are summerized in the following table.

35 MODIFIED RODNAN SKIN SCORE
Systemic Sclerosis All patients with scleroderma have certain shared features of the illness which include: Progressive scarring of blood vessels beginning in the fingers but with risk of extending to internal organs Scarring of tissues outside the blood vessels including skin thickening and lung involvement Varying levels of tissue inflammation and evidence of an overactive immune system The illness is classified in large measure based on the extent and severity of skin thickening. This is usually done by a simple clinical examination technique called the MODIFIED RODNAN SKIN SCORE This individual has skin thickening above the elbows and knees and on the chest and would be classified as having systemic sclerosis with diffuse scleroderma. An individual with skin thickening restricted to the face, forearms, hands and fingers would be said to have systemic sclerosis with limited scleroderma. This clinical picture used to be called the “CREST syndrome”.

36 Classification of Scleroderma Cleveland clinic Center for continuing education
Localized Scleroderma (Localized cutaneous fibrosis) Limited or generalized morphea: Circumscribed patches of sclerosis Linear scleroderma: Linear lesions seen in childhood En coup de sabre: Linear lesions of the scalp or face Systemic Scleroderma (Cutaneous and noncutaneous involvement) Limited cutaneous systemic sclerosis (lcSSc), formerly called CREST syndrome (calcinosis of the digits, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) Diffuse cutaneous systemic sclerosis (dcSSc): Sclerosis of proximal extremities, trunk, and face Systemic sclerosis sine scleroderma (ssSSc): Organ fibrosis only; no skin thickening Subsets of Systemic Sclerosis Diffuse Cutaneous Systemic Sclerosis (dcSSc) Onset of Raynaud's within 1 year of onset of skin changes (puffy or hidebound skin) Truncal and acral skin involvement Presence of tendon friction rubs Early and significant incidence of interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement Absence of anticentromere antibodies Nailfold capillary dilation and capillary destruction Antitopoisomerase antibodies (30% of patients) Limited Cutaneous Systemic Sclerosis (LcSSc) Raynaud's phenomenon for years (occasionally decades) Skin involvement limited to hands, face, feet, and forearms (acral) or absent A significant late incidence of pulmonary hypertension, with or without interstitial lung disease, trigeminal neuralgia, skin calcifications, telangiectasias A high incidence of anticentromere antibodies (70%-80%) Dilated nailfold capillary loops, usually without capillary dropout

37 Septal infarction pattern Ventricular conduction abnormalities
Major Clinical Manifestations of Systemic SclerosisCutaneous Diffuse edema of hands and feet (early stages) Progressive skin tightening Sclerodactyly Calcinosis Telangiectasias Digital ulcers and pits Contractures Hyperpigmentation, hypopigmentation, salt and pepper skin Characteristic facies Vascular Raynaud's phenomenon Nailfold capillary changes Digital ischemia and ulcers Vasculitic leg ulcers (rare) Pulmonary Interstitial lung disease, including alveolitis and interstitial fibrosis Pulmonary hypertension Recurrent aspiration pneumonitis caused by esophageal reflux and dysmotility Chest wall restriction (decreased thoracic compliance) Respiratory muscle weakness Cardiac Cardiomyopathy (systolic and diastolic dysfunction): Congestive heart failure Conduction defects Septal infarction pattern Ventricular conduction abnormalities Arrhythmias Heart blocks Pericarditis or pericardial effusion (impending renal crisis) Renal Scleroderma renal crisis (hypertension, renal failure MAHA) Musculoskeletal and Rheumatologic Arthralgia Tendon friction rubs (relatively specific for diffuse scleroderma) Inflammatory arthritis, erosive arthropathy (rare) Myopathy, myositis Gastrointestinal Gastroesophageal reflux Esophageal dysmotility, aperistaltic esophagus Esophageal stricture Adenocarcinoma arising in Barrett's esophagus (occasionally) Watermelon stomach (gastric antral vascular ectasias [GAVE]): Iron-deficiency anemia Decreased peristalsis throughout the GI tract, leading to bloating, early satiety, stasis, and pseudo-obstruction Bacterial overgrowth and malabsorptive diarrhea, alternating diarrhea and constipation Megacolon (rare) Colonic wide-mouth diverticuli (usually asymptomatic) Pneumatosis cystoides intestinales Primary biliary cirrhosis Anal incontinence Endocrine Hypothyroidism Neurologic Carpal tunnel syndrome Trigeminal neuralgia

38 Major Clinical Manifestations of Systemic Sclerosis
Clinical manifestations of scleroderma. A, Generalized morphea. B, Diffuse edema of hands. C, Firm, thickened skin. D, Flexion contractures of fingers. E, Raynaud's phenomenon (pallor Clinical manifestations of scleroderma. F, Ischemic digital ulcer. G, Telangectasias on the face (a), dorsum of the hand (b), mucosa (c). H, Calcinosis cutis

39 Bibasilar pulmonary fibrosis
American College of Rheumatology Diagnostic Criteria for Systemic Sclerosis American College of Rheumatology Diagnostic Criteria for Systemic Sclerosis Major Criterion Proximal sclerodermatous skin changes (proximal to the metacarpophalangeal joints) Minor Criteria* Sclerodactyly Digital pitting scars of fingertips or loss of substance of the distal finger pads Bibasilar pulmonary fibrosis *The patient should fulfill the major criterion or two of the three minor criteria. Adapted from Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980;23:

40 ACR : American College of Rheumatology AULAR : European League Against Rheumatism

41 High-resolution computed tomography scan of the chest
High-resolution computed tomography scan of the chest.A, Ground-glass opacification. B, Coarse reticulation and honeycombing.

42 Systemic L E. ACR mnemonic of SLE diagnostic criteria Testing
The following are the ACR diagnostic criteria in SLE, presented in the "SOAP BRAIN MD" mnemonic: Serositis Oral ulcers Arthritis Photosensitivity Blood disorders Renal involvement Antinuclear antibodies Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies) Neurologic disorder Malar rash Discoid rash Testing The following are useful standard laboratory studies when SLE is suspected: CBC with differential Serum creatinine Urinalysis with microscopy Other laboratory tests that may be used in the diagnosis of SLE are as follows: ESR or CRP results Complement levels Liver function tests Creatine kinase assay Spot protein/spot creatinine ratio Autoantibody tests Imaging studies The following imaging studies may be used to evaluate patients with suspected SLE: Joint radiography Chest radiography and chest CT scanning Echocardiography Brain MRI/ MRA Cardiac MRI Procedures Procedures that may be performed in patients with suspected SLE include the following: Arthrocentesis Lumbar puncture Renal biopsy

43 Systemic L E. ACR mnemonic of SLE diagnostic criteria Testing
The following are the ACR diagnostic criteria in SLE, presented in the "SOAP BRAIN MD" mnemonic: Serositis Oral ulcers Arthritis Photosensitivity Blood disorders Renal involvement Antinuclear antibodies Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies) Neurologic disorder Malar rash Discoid rash Testing The following are useful standard laboratory studies when SLE is suspected: CBC with differential Serum creatinine Urinalysis with microscopy Other laboratory tests that may be used in the diagnosis of SLE are as follows: ESR or CRP results Complement levels Liver function tests Creatine kinase assay Spot protein/spot creatinine ratio Autoantibody tests Imaging studies The following imaging studies may be used to evaluate patients with suspected SLE: Joint radiography Chest radiography and chest CT scanning Echocardiography Brain MRI/ MRA Cardiac MRI Procedures Procedures that may be performed in patients with suspected SLE include the following: Arthrocentesis Lumbar puncture Renal biopsy

44 Systemic L E. ACR mnemonic of SLE diagnostic criteria Testing
The following are the ACR diagnostic criteria in SLE, presented in the "SOAP BRAIN MD" mnemonic: Serositis Oral ulcers Arthritis Photosensitivity Blood disorders Renal involvement Antinuclear antibodies Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies) Neurologic disorder Malar rash Discoid rash Testing The following are useful standard laboratory studies when SLE is suspected: CBC with differential Serum creatinine Urinalysis with microscopy Other laboratory tests that may be used in the diagnosis of SLE are as follows: ESR or CRP results Complement levels Liver function tests Creatine kinase assay Spot protein/spot creatinine ratio Autoantibody tests Imaging studies The following imaging studies may be used to evaluate patients with suspected SLE: Joint radiography Chest radiography and chest CT scanning Echocardiography Brain MRI/ MRA Cardiac MRI Procedures Procedures that may be performed in patients with suspected SLE include the following: Arthrocentesis Lumbar puncture Renal biopsy characteristically spares the nasolabial folds

45 Clinical features of SLE

46

47 Examples of hirsutism

48 Ferriman Gallwey Evaluation of Hirsutism
It is simply a representation of hair growth in a male pattern on a woman shown in four different degrees of severity in 11 different body parts; namely the upper lip, chin, chest, upper back, lower back, upper abdomen, lower abdomen, arm, forearm, thigh, and lower leg. The Ferriman Gallwey scoring system is used to score the degree of excess male pattern body hair . The scorecard of every body location under survey begins from 0 (no excessive terminal hair growth) to 4 (extensive terminal hair growth) and the numbers are added up (=sum of all scores) to a maximum count of 36. While most experts refer to a modified score of 8 or more to diagnose hirsutism . Based on this score pattern and other clinical tests, hirsutism can be evaluated as mild (8 -16), moderate ( ) or more than 25 as severe. Each ethnic group has an upper limit of the normal value Although more objective tools are available (including photographic evaluation, microscopic measurement of hair diameter with extensive counting of shafts, computerized assessment of photographic measures, and others), these are complex, expensive, or difficult to use. The ease of use and low cost of the Ferriman Gallwey system make it a potentially attractive instrument for use in the clinic. In patients with polycystic ovary syndrome (PCOS), it has been shown to validly reflect androgen excess compared with expensive and elaborate objective methods, including microscopic measurement of hair diameter with extensive counting of individual shafts.

49 Ferriman & Gallwey scoring system

50 Ferriman-Gallwey system for clinical scoring of hirsutism
Ferriman-Gallwey system for clinical scoring of hirsutism. Each of the 11 designated body areas is assigned a score of 0 (absence of coarse dark terminal hairs) to 4 (extensive terminal hair growth). A score higher than 8 indicates hirsutism. Hair scores over the forearms and lower leg do not contribute significantly to the distinction of hirsutism from nonhirsutism.

51 Semiquantitative scoring system for hirsuitism

52 Evaluation score sheet used to evaluate women suspected of hirsutism
Evaluation score sheet used to evaluate women suspected of hirsutism. The degree of terminal hair growth is scored in each of nine body areas from 0 to 4, and the scores in each area are summed (if no excess terminal hairs are noted in an area the score is zero and left blank). The presence of acne (using a scale from 1 to 4) and acanthosis is also recorded.

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54 Pemphigus vulgaris

55 Index of Skin and Mucous Involvement in Pemphigus Vulgaris (ISMIPV)
Creation of the clinical score Application of the ISMIPV results in a total score that is based on the evaluation of four different items : @ The number of blisters or erosions where a blister had previously been present (i.e. includes blisters that have already ruptured). Areas of residual post-inflammatory pigmentation should be excluded. Score: points. @ Size of the blisters or erosions. Only the largest visible lesion should be taken into consideration, the greatest diameter of which should be measured and included in the score. If the patient has more than 20% of his/her body area covered with whole or ruptured blisters, this is also reflected in the score. Score: points. @ Nikolsky's sign. This item receives a score if Nikolsky's sign or the Asboe-Hansen sign is positive, both being indicative of the active disease. Score: 0 or 20 points. @ Involvement of mucous membranes and sepsis. For the purpose of scoring, the following sites are considered mucous membranes: eyes, nose, ears, mouth, anus and external genitalia. The number of lesions at each one of these sites is not important, i.e. if the site is affected at all, this counts as one mucous membrane site when adding the score. In this item and for the purpose of calculating the score, only intense and/or persistent restrictions to the affected mucous membrane site are considered to represent severe functional repercussions. Examples: conjunctival synechiae; the patient's inability to feed him/herself and a weight loss >10% since the mucous membrane site was first affected; stenoses of natural orifices; mucous membrane infections with sepsis. Patients with sepsis due to skin lesions also receive a maximum score in this item even if they have no visible mucous membrane involvement. Score: points. Total ISMIPV score is the result of the sum of the scores awarded in the four items outlined above and may range from 0 to 100 points, grouped into 5-point intervals (possible ISMIPV scores: 0, 5, 10, 15, 20, 25, 30, 35, 40, 45, 50, 55, 60, 65, 70, 75, 80, 85, 90, 95 and 100, i.e. there are 21 possible scores).

56 Index of Skin and Mucous Involvement in Pemphigus Vulgaris (ISMIPV)

57 Pemphigus area and activity score (PAAS) _It is a specific scoring system that has been suggested by Agarwal et a]  for the clinical assessment of severity and progression of pemphigus vulgaris. PAAS is calculated separately for cutaneous and mucus membrane lesions . Total score is calculated by adding up the cutaneous score and the mucous membrane score.

58 Pemphigus Activity Score introduces intensity of steroid and immunosuppressive therapy along with the extent of disease. But it suffers from a lack of differential clinical involvement of mucosal and cutaneous lesions and an insensitive quantification of lesions.

59 Pemphigus Disease Area Index (PDAI) integrates cutaneous with mucosal disease in well-defined anatomical locations, assesses number and sizes of lesions and also scores post-inflammatory hyperpigmentation of resolving lesions

60 Visual analog scale for evaluation of pruritus

61 Autoimmune Bullous Skin Disorder Intensity Score is a quality- and quantity-based score for cutaneous and oral mucosal lesions. This system claims to monitor the clinical status of individual patients over time vis-à-vis inter-patient differentiation assessed by other systems. In addition, this system can be used for assessing autoimmune diseases other than pemphigus alone, and thus is more versatile

62 Saraswat's oral pemphigus scoring (2OO3)
As mucosal lesions in pemphigus often predate cutaneous lesions, are more recalcitrant and recurrent, associated with severe morbidity (halitosis, dysphagia), often improve with reducing depth and not necessarily by reducing count or size, defy BSA assessment and behave differently from cutaneous lesions, an independent scoring system was devised for oral pemphigus [Table ]. It is reportedly useful for other diseases, viz., benign mucosal pemphigoid, herpetic gingivostomatitis and Stevens Johnson syndrome, and eliminates subjective inter-observer variability. The versatility of this system is revealed by the fact that ABSIS system has also borrowed from this scoring system.

63 Mahajan's scoring system ( 2OO5) The search for perfect system is still on

64 Kumar's scoring system (2OO6) The search for perfect system is still on

65 Harman's pemphigus grading (2O11) The search for perfect one is still on

66 Bullous Pemphigoid Disease Area Index (BPDAI): Objective measure of cutaneous and mucosal involvement in bullous pemphigoid Murrell et al. 2O12

67 Autoimmune Bullous Skin Disorder Intensity Score (ABSIS): Objective measure of clinical activity that can be used for bullous pemphigoid. BSA indicates body surface area Frutze et al. 2OO7

68 Accepted Evaluation Criteria for Bullous Pemphigoid (BP).Murrell et al. 2O12  
Early observation points Baseline Day that BP therapy is started by physician. Control of disease activity Time at which established lesions begin to heal or pruritic symptoms start to abate and new lesions cease to form. Time to control of disease activity Time interval from baseline to control of disease activity. End of consolidation phase Time at which no new lesions have developed for minimum of 2 wk and approximately 80% of lesions have healed and pruritic symptoms are minimal. Transient lesions New lesions that heal within 1 wk or pruritus lasting < 1 wk and clearing without treatment. Nontransient lesions New lesions that do not heal within 1 wk or pruritus continuing > 1 wk with or without treatment. Complete remission during tapering Absence of nontransient lesions while patient is receiving more than minimal therapy. Late observation points Minimal therapy ≤ 0.1 mg/kg/d of prednisone (or equivalent) or 20 g/wk of clobetasol propionate and/or minimal adjuvant or maintenance therapy. Minimal adjuvant therapy and/or maintenance therapy Following doses or less: methotrexate 5 mg/wk; azathioprine 0.7 mg/kg/d (with normal thiopurine s-methyltransferase levels); mycophenolate mofetil 500 mg/d; mycophenolic acid 360 mg/d; or dapsone 50 mg/d. Partial remission on minimal therapy Presence of transient new lesions that heal within 1 wk while patient is receiving minimal therapy for at least 2 mo. Complete remission on minimal therapy Absence of new transient or established lesions or pruritus while patient is receiving minimal therapy for at least 2 mo. Partial remission off therapy Presence of transient new lesions that heal within 1 wk without treatment while patient is off all BP therapy for at least 2 mo. Complete remission off therapy Absence of new or established lesions or pruritus while patient is off all BP therapy for at least 2 mo. Mild new activity < 3 lesions/mo that do not heal within 1 wk, or extension of established lesions or pruritus once a wk but less than daily in a patient who has achieved disease control; these lesions have to heal within 2 wk. Relapse/flare Appearance of ≥ 3 new lesions/mo or at least 1 large (> 10 cm diameter) lesion that does not heal within 1 wk, or extension of established lesions or daily pruritus in patient who has achieved disease control. Failure of therapy for initial control Development of new nontransient lesions or continued extension of old lesions, or failure of established lesions to heal or continued pruritus despite: clobetasol propionate 40 g/d for 4 wk; prednisone 0.75 mg/kg/d or equivalent for minimum of 3 wk with or without adjuvant therapy; a tetracycline on full dosing for 4 wk. Dapsone 1.5 mg/kg/d for 4 wk; methotrexate 15 mg/wk (if > 60 kg and no renal impairment) for 4 wk; azathioprine 2.5 mg/kg/d for 4 wk (if thiopurine s-methyltransferase levels are normal); mycophenolate mofetil 40  mg/kg/d (if normal renal function) for 4 wk.

69 Melasma

70 Melasma area and severity index. MASI SCORE IJDVL 78: 165, 2O12
  Melasma  Melasma area severity index (MASI) is developed by Kimbrough-Green et al for the assessment of melasma.  The severity of the melasma in each of the four regions (forehead, right malar region, left malar region and chin) is assessed based on three variables: percentage of the total area involved (A), darkness (D), and homogeneity (H). A numerical value assigned for the corresponding percentage area involved is as follows: 0=no involvement; 1=<10% involvement; 2=10-29% involvement; 3=30-49% involvement; 4=50-69% involvement; 5=70-89% involvement; and 6=90-100% involvement. The darkness of the melasma (D) is compared to the normal skin and graded on a scale of 0 to 4 as follows: 0=normal skin color without evidence of hyperpigmentation; 1=barely visible hyperpigmentation; 2=mild hyperpigmentation; 3=moderate hyperpigmentation; 4=severe hyperpigmentation. Homogeneity of the hyperpigmentation (H) is also graded on a scale of 0 to 4 as follows: 0=normal skin color without evidence of hyperpigmentation; 1=specks of involvement; 2=small patchy areas of involvement <1.5 cm diameter; 3=patches of involvement >2 cm diameter; 4=uniform skin involvement without any clear areas). To calculate the MASI score, the sum of the severity grade for darkness (D) and homogeneity (H) is multiplied by the numerical value of the areas (A) involved and by the percentages of the four facial areas (10-30%).  Total MASI score: Forehead 0.3 (D+H)A + right malar 0.3 (D+H)A + left malar 0.3 (D+H)A + chin 0.1 (D+H)A

71

72     Dyshidrotic eczema  Dyshidrotic eczema area and severity index (DASI) is proposed for dyshidrotic eczema. Dyshidrotic eczema area and severity index (DASI) Based on the severity grade of single items - number of vesicles per square centimetre (V), erythema (E), desquamation (S) and itch (I) - and the extension of the affected area (A) and is calculated with defined score points (p) as: DASI = (pV + pE = pS + pI) ´ pA. DASI was found to be a simple and useful tool to assess the severity of dyshidrotic eczema and the effect of therapy. Thus application of mind helps to design scores for semi-objective assessment of skin diseases. Till better objective parameters are developed, scores will continue to remain the gold standard for assessing the severity of dermatological diseases in clinical research.

73 Scoring systems in acne vulgaris IJDVL 75:323, 2OO(
. The two commonly used measures are grading and lesion counting. Grading is a subjective method, which involves determining the severity of acne, based on observing the dominant lesions, evaluating the presence or absence of inflammation and estimating the extent of involvement. Lesion counting involves recording the number of each type of acne lesion and determining the overall severity.. . Individual methods Several systems for grading the severity of acne currently exist. @ In 1956, Pillsbury, Shelley and Kligman published the earliest known grading system. The grading includes the following: Grade 1: Comedones and occasional small cysts confined to the face. Grade 2: Comedones with occasional pustules and small cysts confined to the face. Grade 3: Many comedones and small and large inflammatory papules and pustules, more extensive but confined to the face. Grade 4: Many comedones and deep lesions tending to coalesce and canalize, and involving the face and the upper aspects of the trunk. @ In 1958, James and Tisserand in their review of acne therapy, provided an alternative grading scheme Grade 1: Simple non-inflammatory acne - comedones and a few papules. Grade 2: Comedones, papules and a few pustules. Grade 3: Larger inflammatory papules, pustules and a few cysts; a more severe form involving the face, neck and upper portions of the trunk. Grade 4: More severe, with cysts becoming confluent. The response to acne therapy could never be precisely assessed by grades of 1 to 4 and such classification systems are overly simple. @ In 1977, Michaelson et al., counted the number of lesions on the face, chest and back. They gave a different score to each lesion type. Comedones were valued at 0.5; papules, at 1.0; pustules, at 2.0; infiltrates, at 3.0; and cysts, at 4.0. By multiplying the number of each type of lesion by its severity index and adding each product, these authors obtained a total score that represented the severity of the disease for each visit. This grading system has been criticized on the grounds that scores ascribed to lesions are non-parametric, whereas absolute counts are a parametric data and it is probably wrong to mix these two types of data. @ In 1979, Cook, et.al. evaluated the overall severity of acne on a 0-8 scale anchored to photographic standards that illustrate grades 0, 2, 4, 6 and 8 . In addition to the photographic standards, a nine-point scale for comedones, papules and macules over the face was used in conjunction for more sensitivity. @ In 1984, Burke & Cunliffe ., presented the Leeds technique. They described two scoring systems. The first is an overall assessment of acne severity for use in routine clinic and the second, a counting system for detailed work in therapeutic trials. A scale of 0 (no acne) to 10 (the most severe) was used for grading. The groups 0 to 2 were divided into subgroups, by 0.25 divisions. Grades 0.25 to 1.5 represented patients with physiological acne or "acne minor" and those with grades of 1.5 or more have clinical acne or "acne major." @ In 1996, Lucky et al ., assessed the reliability of acne lesion counting. Acne counts were recorded on a template divided into five facial segments: Right and left sides of the forehead, right and left cheeks and chin. The nose and the area around it were excluded. Counts of each lesion type were recorded within each segment of the template. Total lesion count, along with total inflammatory lesions and comedonal counts, were then calculated. They concluded that reliability of acne lesion counting was excellent when performed by the same trained rater over time. @ In 1997, Doshi et al. devised a global acne grading system (GAGS). This system divides the face, chest and back into six areas (forehead, each cheek, nose, chin and chest and back) and assigns a factor to each area on the basis of size. @ In 2008, Hayashi et al. , used standard photographs and lesion counting to classify acne into four groups. They classified acne based on the number of inflammatory eruptions on half of the face as 0-5, "mild"; 6-20, "moderate"; 21-50, "severe"; and more than 50, "very severe." Other grading systems used for grading acne vulgaris are summarized in the . Acne vulgaris was graded by Indian authors, using a simple grading system, which classifies acne vulgaris into four grades as follows. Grade 1: Comedones, occasional papules. Grade 2: Papules, comedones, few pustules. Grade 3: Predominant pustules, nodules, abscesses. Grade 4: Mainly cysts, abscesses, widespread scarring.

74 In 1979, Cook et al, evaluated the overall severity of acne on a 0-8 scale anchored to photographic standards that illustrate grades 0, 2, 4, 6 and 8 . In addition to the photographic standards, a nine-point scale for comedones, papules and macules over the face was used in conjunction for more sensitivity

75 In 1997, Doshi, Zaheer and Stiller devised a global acne grading system (GAGS). This system divides the face, chest and back into six areas (forehead, each cheek, nose, chin and chest and back) and assigns a factor to each area on the basis of size

76 In 2008, Hayashi et al. used standard photographs and lesion counting to classify acne into four groups. They classified acne based on the number of inflammatory eruptions on half of the face as 0-5, "mild"; 6-20, "moderate"; 21-50, "severe"; and more than 50, "very severe." Other grading systems used for grading acne vulgaris are summarized in the table

77 Some of scoring system for assessment of acne vulgaris

78 Investigator's Global Assessment (IGA) for acne vulgaris

79 Acne (e. g. , severity ) may also be measured (e. g
Acne (e.g., severity ) may also be measured (e.g., graded) using a facial evaluation scoring system

80 Cardiff Acne Disability Index (CADI)

81 Facial Cutaneous Evaluation after treatment of acne vulgaris

82 Adolescents with acne filled all the questions including the Children Dermatology Life Quality Index.An Bras DCerm87 no.6, 2O12

83 We used the Skin Of Mine Acne module at skinofmine
We used the Skin Of Mine Acne module at skinofmine.com to quantify acne of six individuals, where the severity of the acne of the individuals ranged from none to severe.  In each case, the computer mouse was used to manually highlight the areas of concern for each individual.  Results are shown below. The lower row of images shows the highlighted regions in green.

84

85 Items of interest in the clinical history of patients with chronic spontaneous urticaria

86 Urticaria activity score (UAS) The UAS consisted of the sum of the wheal number score and the itch severity score. The wheal numbers are graded from 0 to 3 as follows: 0 - less than 10 small wheals (diameter, < 3 cm); to 50 small wheals or less than 10 large wheals (diameter, > 3 cm); 2 - greater than 50 small wheals or 10 to 50 large wheals; and 3 - almost the whole body is covered. The severity of the itching is graded from 0 to 3 (0, none; 1, mild; 2, moderate; and 3, severe).

87 placing four circles on top of each other
placing four circles on top of each other. First circle is a knob to move circle. Second circle is divided in to four parts for wheal score from 0 to three. Third circle is for assessing pruritus severity score from 0 to three. Last and outermost circle is total of wheal score and pruritus score Ind J Derm. 57 :41O ,2O12

88 AN Bras, Derm, 8O :no. 6, 2OO5

89 AN Bras, Derm, 8O :no. 6, 2OO5

90 AN Bras, Derm, 8O :no. 6, 2OO5

91 AN Bras, Derm, 8O :no. 6, 2OO5

92 AN Bras, Derm, 8O :no. 6, 2OO5

93 Provocation testing for physical and cholinergic urticarias

94 Threshold testings for physical urticarias

95 Salient differences between female pattern hair loss and chronic telogen effluvium
Summary of miscellaneous topical therapies tried in female pattern hair loss

96 Classification of methods to evaluate hair loss

97 Evaluation techniques for hair loss

98 Skinfold measurements can give a clue to your hormone levels.

99 Calculate body surface area burned
To estimate scattered burns: patient's palm surface = 1% total body surface area

100 The Rape of Proserpina 1621-22 Marble
The Rape of Proserpina Marble. height 295 cm Galleria Borghese, Rome


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