Recording Site and Severity of Oral Pemphigus Vulgaris With New Tool

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ODSS is easy to use and facilitates detailed recording of both the site and the severity of pemphigus vulgaris lesions.
ODSS is easy to use and facilitates detailed recording of both the site and the severity of pemphigus vulgaris lesions.

The Oral Disease Severity Score (ODSS) is a valid and reproducible tool for recording oral pemphigus vulgaris (PV) disease activity, with interobserver and intraobserver reliability that is superior to that of the Pemphigus Disease Area Index (PDAI), the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and the Physician's Global Assessment (PGA) for the detection of oral lesions, according to the results of a recent UK study published in the British Journal of Dermatology.

A total of 15 patients with a mean age of 56±14.2 years (range, 23-77 years) with a confirmed diagnosis of predominantly oral PV participated in the study. Overall, 13 patients had mild to moderately severe oral disease; in 1 patient, disease was in clinical remission; and 1 had severe oral disease.

All participants were receiving systemic treatment — mycophenolate mofetil, azathioprine, or rituximab with or without prednisolone — at the time of the study.

The comprehensive scoring system of the ODSS divides the mouth into 17 sites that are weighted according to area of possible involvement and allocated a site score of 0 to 2. The sites include the outer and inner lips; left and right buccal mucosa 6 gingival segments; hard palate (left, right, or both); soft plate (left, right, or both); dorsum tongue (left, right, or both); left ventral tongue, right ventral tongue, floor of mouth (left, right, or both); and oropharynx. Individual sites are assigned an activity score of 0 to 3, with mild inflammation=1, moderate inflammation=2, and ulceration=3.

A subjective assessment of the patient's oral pain in the prior week is also included (verbal rating scale of 0 to 10). The theoretic maximum score is 106; however, >95% of patients are expected to have scores in the range of 0 to 60, which represents a clinical range from remission to severe disease.

Interobserver reliability was evaluated using an intraclass coefficient (ICC). For the ODSS total score, the ICC was 0.83 compared with an ICC of 0.79 on the PDAI (oral total activity), ICC of 0.71 on the ABSIS (oral total), and ICC of 0.7 on the PGA.

Intraobserver agreement between the initial scoring and rescoring of the same participant by 2 different clinicians revealed an ICC for each of 0.97 and 0.96 for ODSS total score, 0.99 and 0.82 for PDAI oral activity, 0.86 and 0.45 for ABSIS oral total, and 0.99 and 0.64 for the PGA. Convergent validity was good, with a correlation coefficient of >0.5 (P <.0001).

The mean time needed to complete each scoring system was as follows: ODSS= 76±37 seconds; PDAI=117±16 seconds; and ABSIS=75±19 seconds.

The investigators concluded that the ODSS is easy to use and facilitates detailed recording of both the site and the severity of PV lesions, including those that are healing. They suggest that this scoring tool would be a useful addition for future multicenter studies, as well as for recording sequential disease activity in the clinic.

Reference

Ormond M, McParland H, Donaldson ANA, et al. An oral disease severity score (ODSS) validated for use in oral pemphigus vulgaris [published online January 3, 2018]. Br J Dermatol. doi: 10.1111/bjd.16265

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