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
Cite this article: Srinivasan S, Mathumathy R. Scoring systems in bullous dermatoses. Our Dermatol Online. 2013; 4(Suppl.3): 663-665
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.
Comments are closed.