A proposal of a comprehensive mucocutaneous activity index for Behçet’s disease

Dear Editor,

Behçet’s disease (BD) is an inflammatory multi-system disorder causing recurrent oral, genital ulcers, skin lesions including acne-like eruptions and erythema nodosum-like lesions, and uveitis, in addition to neurological and gastrointestinal manifestations. However, no specific biochemical or serological marker is currently present for the evaluation of disease activity in BD [1, 2]. In addition, no standardized mucocutaneous activity index is currently present to monitor clinical manifestations with mucocutaneous lesions [3, 4]. The present manuscript outlines a mucocutaneous activity index we developed (Figure 1).

Figure 1

Figure 1. Mucocutaneous activity index in Behçet’s disease.

The mucocutaneous activity index consists of five elements [oral ulcer score, genital ulcer score, acne-like lesion score, erythema nodosum-like lesion score, and numerical rating scale (NRS) of pain with mucocutaneous lesions]. Each lesion type is scored based on the count and size of the lesions over the last 4 weeks. To determine the oral ulcer score and genital ulcer score, doctors ask a patient how many ulcers and what size ulcers they had in their mouth over the last 4 weeks. To determine the acne-like skin lesion score, doctors ask a patient how many acne or acne-like skin lesions they had in their mouth over the last 4 weeks. To determine the erythema nodosum-like lesion score, doctors ask a patient how many erythema nodosum-like lesions and what size they had in their mouth over the last 4 weeks. To measure the NRS of pain associated with the mucocutaneous lesions, doctors ask a patient how much pain they feel associated with their conditions in their mouth over the last 4 weeks.

The clinical data of the BD patients from our dermatology department between June and July 2023 were scored according to the mucocutaneous activity index (Supplementary Table S1) to confirm whether the index could reflect clinical conditions. Nine patients were seen in our division, all of whom were female and had a median age of 42.6 years. We determined that there was moderate disease activity in six patients and mild disease activity in three patients. A 10-point NRS is a simple rating system that is commonly used globally in oral medicine to assess various diseases, such as atopic dermatitis. We found significantly positive correlations between the total score and the NRS of pain in the nine patients with BD (r = 0.885, p = 0.0015) (Supplementary Figure S1). We found significantly positive correlations between the total score and numbers of oral ulcer in them (p = 0.0071). Similarly, positive significant correlations were observed for the total score and size of oral ulcer in them (p = 0.0192). Based on this correlation, we suggest that the mucocutaneous activity index could play some role in helping the decision-making process for treatment strategies in BD patients with mucocutaneous lesions. In conclusion, we here propose a comprehensive mucocutaneous activity index for Behçet’s disease that is useful for daily practice and evaluating therapeutic efficacy in clinical trials. We are currently validating the index in a large-scale study using the Japanese Behçet’s disease registry.

Data availability statement

The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.

Ethics statement

This study was approved by the Tohoku Medical and Pharmaceutical University Ethics Committee, and informed consent was obtained from all patients (No. 2021-2-045).

Author contributions

All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.

Funding

This study was supported by a grant for the Behçet’s Disease Research Committee from the Japanese Ministry of Health, Labour and Welfare (Grant no. 23FC0201).

Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Supplementary material

The Supplementary Material for this article can be found online at: https://www.frontierspartnerships.org/articles/10.3389/jcia.2024.12652/full#supplementary-material

SUPPLEMENTARY TABLE S1 | Mucocutaneous activity index in Behçet’s disease.

SUPPLEMENTARY FIGURE S1 | We found significantly positive correlations between total score (X-axis) and NRS, of pain (Y-axis) in these patients (r = 0.885, p = 0.0015).

References

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