2013.4-3s.Scoring

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Our Dermatol Online.  2013; 4(Suppl. 3): 663-665
DOI:.  10.7241/ourd.20134.160
Date of submission:  Date of submission: 01.08.2013 / acceptance: 21.08.2013
Conflicts of interest: None
 

SCORING SYSTEMS IN BULLOUS DERMATOSES

Sunderamoorthy Srinivasan, Ramamoorthy Mathumathy

Chettinad Hospital & Research Institute, Kelambakkam, Chennai, India
 

Corresponding authors:  Prof. Sunderamoorthy Srinivasan, Dr. R. Mathumathy    e-mail: hamsrini@yahoo.co.in, drmathumathy@yahoo.co.in


 

Sir
These group of disorders are chronic blistering auto immune disorders characterized histologically by intra epidermal/sub epidermal blister formation. They have circulatory auto antibodies against distinct adhesion molecules of epidermis and BMZ. They are potentially life threatening, characterized by widespread blisters and erosions of skin and mucosa. Pemphigus being clinically heterogenous group of disorder, comprehensive scoring systems are needed to assess the severity of disease for effective monitoring of treatment. Pemphigus Disease Area Index (Tabl. I) 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. Numerous scoring systems (Tabl. II-IV) have been described in literature at different times to score disease activity of pemphigus.
 
SCORING SYSTEMS

ABSIS
(Auto immune bullous skin disorder intensity score)
 
SKIN INVOLVEMENT
2 parameters
* The extent of affected area (calculated by rule of nine)
* Quality of skin lesions
 
% of BSA X weighing factor
Weighing factor
1.5- for erosive,exudative lesions, blisters, positive Nikolsky sign. 1
– for erosive, dry lesions
0.5- re epithelialized lesions
0-150 points
Compare scores from initial scores with treatment to monitor disease activity.
 
ORAL INVOLVEMENT
1st Mucosal score-extent of lesions (0-11)
 
1- upper gingival mucosa
2-lower gingival mucosa
3-upper lip mucosa
4-lower lip mucosa
5-left buccal mucosa
6-right buccal mucosa
7-tongue
8-floor of mouth
9-hard palate
10-soft palate
11-pharynx
 
Rating
0-no lesion
1-presence of lesion
 
2nd Mucosal involvement- severity of lesion (0-45)
 
1 point-if pain/bleeding always occurred
0.5 point-pain/bleeding occurred sometimes
0 point-no pain
 
Types of food
Water -1*x
Soup -2*x
Yoghurt -3*x
Custard -4*x
Smashed potatoes/scrambled egg -5*x
Baked fish -6*x
White bread -7*x
Apple/raw carrot -8*x
Whole grain bread -9*x
 
Skin
Activity

Damage
PDAI-skin



Anatomical
location
Erosion/blister or new erythema
Number of lesions, if<3
Post-inflamatory hyperpigmentation or erythema from
resolving lesions
Anatomical
location
0 = Absent
1 = 1-3 lesions, up to one?2cm in any diameter,
none >6cm
2 = 2-3 lesions at laest two >2cm, none >6cm
3 = >3 lesions, none >6cm
5 = >3 lesions, and/or at least one >6cm
10 = >3 lesions, and/or at least one >16cm or
entire area
Number of lesions, if<3
0 = Absent
1 = Present
Ears
Nose
Rest of face
Neck
Chest
Andomen
Back/buttocks
Arms
Hands
Legs
Feet
Genitals
Total skin
/120
Number of lesions, if<3 /12
PDAI-skin



Scalp
Erosion/blister or new erythema
Number of lesions, if<3
Post-inflamatory hyperpigmentation or erythema from
resolving lesions
Total scalp
0 = Absent
1 = In one quadrat
2 = Two quadrats
3 = Three quadrats
4 = Whole skull
10 = At least one lesion ?6cm
Number of lesions, if<3
0 = Absent
1 = Present
Total scalp
/10
Number of lesions, if<3
/1
PDAI-mucous membranes



Anatomical
location
Erosion/blister
0 = Absent
1 = 1 lesion
2 = 2-3 lesions
5 = >3 lesions or 2 lesions >2cm
10 = entire area
Number of lesions, if<3

Eyes
Nose
Buccal mucosa
Hard palate
Soft palate
Upper gingiva
Lower gingiva
Tongue
Floor of mouth
Labial mucosa
Posterior pharynx
Anogenital
Total mucosa
/120
Number of lesions, if<3

               Table I. PDAI (Pemphigus disease activity index).

Severity

Antibody Titer Level
Oral
Skin
Antibody Titer Level
0 = Nil
0 = Nil
Dsg1 and Dsg3 levels as assessed by ELISA
1 = <3 Erosions
1 = <5 Erosions
Dsg1 and Dsg3 levels as assessed by ELISA
2 = 3-4 Erosions
2 = 5-20 Erosions
Dsg1 and Dsg3 levels as assessed by ELISA
3 = >10 Erosions
3 = >20 Erosions
Dsg1 and Dsg3 levels as assessed by ELISA

               Table II. Harman’s Pemphigus Grading.

Oral score
0 – No mucosal involvement
1 – Minimal disease (only bucal mucosa, labiogingival, lingual, palatal, pharyngeal)
2 – Moderate disease (bucal and labiogingival, lingual, palatal or pharyngeal)
3 – Severe disease (extensive oral erosions, i.e., >3 mucosal sites affected)
Skin score
0 – Quescent disease
1 – Minimal disease (<10% BSA)
2 – Moderate disease (11-30% BSA)
3 – Severe disease (>30% BSA)

               Table III. Kumar’s Scoring System.

Severity
Cutaneus involvement
Mucosal involvement
Mild (1+)
10% BSA, able to carry daily routine without discomfort
or localisation to oral mucosa only
Only localised to buccal mucosa, no difficulty in swallowing or
chewing
Moderate (2+)
10-25% BSA along with oral mucosal involvement, able
to carry out daily routine with discomfort
Buccal and gingivolabial mucosal involvement, difficulty in solid
food intake
Severe (3+)
25-50% BSA along with oral mucosal involvement,
unable to carry out daily routine
Extensive oral mucosal involvement, difficulty in semisolid food
inake
Extensive (4+)
50% BSA along with mucosal involvement, bedridden or
has complications
Extensive oral mucosal involvement, other mucosal involvement,
difficulty in swallowing liquids also (unable to take anything
orally)

               Table IV. Mahajan’s Scoring System.


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