A new grading system for oral pemphigus

Correspondence

Oxford, UK International IJD Blackwell 0011-9059 41 Publishing Science, Journal 2002 Ltd. of Dermatology

Correspondence

A new grading system for oral pemphigus

In almost all patients with pemphigus vulgaris and pemphigus vegetans, oral mucosal lesions occur at some time in the course of the disease, and in 50 –70% of cases these lesions are the presenting complaint.1 Mucosal lesions often precede skin lesions by several months and are very recalcitrant, often taking much longer to heal than skin lesions and frequently recurring, although skin lesions are controlled.2 Oral and pharyngeal erosions also contribute significantly to morbidity, often making eating and drinking difficult or impossible. For these reasons, it is important to have a valid and reproducible grading system to assess the severity of oral mucosal involvement in pemphigus. Such a system would be valuable for accurate evaluation of severity and interstudy comparison of drug efficacy. Most grading systems proposed so far depend upon the number of oral erosions, e.g. the widely used system of Ishii et al.3 and the score used by Harman et al.4 Others have proposed a combination of the subjective assessment of severity and the number of sites involved.5 In the course of evaluating oral pemphigus lesions in more than 100 patients at our clinic, we have noticed certain shortcomings in these methods of assessment. Because oral erosions, even more than skin lesions, tend to extend and coalesce, a count of these lesions is often not a true reflection of disease severity or activity. Often, erosions on gingival margins (desquamative gingivitis) extend across many adjacent teeth forming a continuous eroded margin, thus defying any lesion-count-based assessment. Additionally, when mucosal erosions improve, they become smaller and more superficial without any change in their number. Subjective assessments of severity (mild /moderate /severe) as advocated by Agarwal et al.5 and percentage-area-based grading have the obvious drawback of wide inter-observer variation. We have also noticed that objective physician-based assessments are often insensitive to significant changes in the severity of symptoms, i.e. odynophagia, bleeding, excessive salivation and halitosis. Many patients show significant improvement in their ability to eat solid foods or a marked reduction in overall odynophagia without significant change in the apparent size and severity of lesions. This is because healing erosions are often clinically visible for several days even when they are minimally symptomatic. Also, even isolated erosions over the soft palate or pharynx cause much more severe symptoms than comparatively extensive gingival or buccal lesions. Taking into account the above observations, we have attempted to devise a simple yet rigorous grading system for © 2003 The International Society of Dermatology

oral pemphigus, which incorporates objective and subjective parameters with minimal potential for inter-observer variation. The objective assessment determines the extent of mucosal involvement and includes the evaluation of 11 anatomical sites in the oral cavity and oropharynx. These are the labial mucosa (upper and lower), gingival mucosa (upper and lower), buccal mucosa (left and right), lingual mucosa (dorsal and ventral, including the floor of the mouth), palate (hard and soft, including uvula) and pharynx. The presence or absence of lesions at all these sites is scored as 1 or 0, respectively, and the total score, ranging from 0 to 11, is arrived at by adding all site scores. The second assessment measures the severity of symptoms and is based on a modification of the objectively validated dysphagia grading system of Dakkak and Bennett.6 This score combines information about pain and/or bleeding during eating or drinking of nine types of solid and liquid foods and ranges from 0 (no symptoms) to 45 (severe symptoms) (Table 1). We have devised a list of Indian food equivalents for some categories to make the score applicable to our patients. Further modifications according to local food habits can be developed and tested for use in other countries. The two scores are assessed and recorded separately and any change in either is compared to baseline values of that score. This allows us to separately assess the impact of various therapies on disease extent and symptom severity. Symptomatic therapies, e.g. local anesthetics or barrier agents, are evaluated by the symptom score alone, whereas diseasedirected therapies, e.g. corticosteroids and other immunosuppressive agents, are evaluated by a combination of both scores. Until now, objective scoring of oral pemphigus has not been given the attention it deserves. It has mostly been lumped together with assessment of skin lesions, which is often inappropriate given its often recalcitrant and sometimes divergent clinical course. Traditional teaching regarding pemphigus advises tapering the dose of corticosteroids when the disease is suppressed by approximately 80%.7 While lesion counting and the ‘rule of 9’ can be used to evaluate cutaneous disease activity and response to treatment, oral lesions are difficult to evaluate in the absence of clear criteria. Even recent innovations in therapy such as the high-dose corticosteroidcyclophosphamide pulse regimen8 have disregarded oral lesions in classifying the phases of response and deciding on the duration of treatment. We expect this scoring system to markedly reduce interobserver variability as subjective evaluation of extent or International Journal of Dermatology 2003, 42, 413 – 414

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Correspondence

Table 1 Oral pemphigus symptom score No.

Food article

Additional Indian food equivalents (without chillies, spices or lemon juice)

1 2 3 4 5 6 7 8 9

Water Milk (or thin soup) Custard (or yoghurt or pureed fruit) Jelly Scrambled egg (or baked beans or mashed potato) Baked fish (or steamed potato or cooked carrot) Bread (or pastry) Apple (or raw carrot) Steak (or pork or lamb chop)

– Thin buttermilk Boiled, mashed, dehusked pulses/lentils Porridge, boiled sago/pulses Steamed gram/corn Baked /lightly fried, well-cooked vegetables Chapatis (unleavened bread) – Mutton or chicken, pear, peanut, almond

The presence of symptoms during eating /drinking each liquid or solid food is first determined and scored. Pain / bleeding always = 1 point, sometimes = 1/2 point, and never = 0 point. If a patient cannot eat a particular food at all, that food and all others listed below it are scored 1 point. A score from 0 (no symptoms) to 45 (severe symptoms) is then determined by multiplying the score for each substance by the adjacent line number, and then adding all nine lines. For instance, if a patient is able to take fluids without any symptoms, and soft solids with occasional pain, but cannot eat bread, meat or raw hard fruits, his symptom score would be (1 × 0) + (2 × 0) + (3 × 0) + (4 × 1/2) + (5 × 1/2) + (6 × 1/2) + (7 × 1) + (8 × 1) + (9 × 1) = 31.5.

severity by the observer is eliminated. The symptom score also does not require any individual assessment of severity by the patient, relying only on the presence and frequency of symptoms on eating and drinking common foods. The whole evaluation can easily be performed within 2 min in an outpatient set-up. This scoring system can also be used for other erosive oral conditions such as benign mucosal pemphigoid, herpetic gingivostomatitis and Stevens Johnson syndrome. An obvious lacuna of this scoring system is the omission of the size of erosions as a parameter. However, great practical difficulty in its consistent evaluation led us to exclude size of erosions from the score. We presume that the severity of large erosions localized to a single or a few sites will be adequately reflected in the second (symptom) score. We are using this scoring system to evaluate patients at our clinic with satisfactory results, and objective validation studies are being initiated. Further experience will allow a reduction in the number of food categories in the symptom score. Multicenter studies on a large number of patients would establish the true validity and reproducibility of this scoring system for oral pemphigus. Abir Saraswat, MD, DNB and Bhushan Kumar, MD, MNAMS Chandigarh, India

International Journal of Dermatology 2003, 42, 413 – 414

References 1 Krain LS. Pemphigus. Arch Dermatol 1974; 110: 862 – 865. 2 Scully C. The oral cavity. In: Champion RH, Burton JL, Burns DA, Breathnach SM, eds. Textbook of Dermatology. Oxford: Blackwell Science 1998, 3083 – 3084. 3 Ishii K, Amagai M, Hall RP, et al. Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with Baculovirusexpressed recombinant desmogleins. J Immunol 1997; 159: 2010 – 2017. 4 Harman KE, Seed PT, Gratian MJ, Bhogal BS, Challacombe SJ, Black MM. The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels. Br J Dermatol 2001; 144: 775 – 780. 5 Agarwal M, Walia R, Kochhar AM, Chander R. Pemphigus Area and Activity Score (PAAS) – a novel clinical scoring method for monitoring of pemphigus vulgaris patients. Int J Dermatol 1998; 37: 158 –160. 6 Dakkak K, Bennett JR. A new dysphagia score with objective validation. J Clin Gastroenterol 1992; 14: 99 –100. 7 Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin 1993; 11: 429 – 452. 8 Pasricha JS, Das SS. Curative effect of dexamethasonecyclophosphamide pulse therapy for the treatment of pemphigus vulgaris. Int J Dermatol 1992; 31: 875 – 879.

© 2003 The International Society of Dermatology