(027) PREVALENCE OF CONDITIONS ASSOCIATED WITH ABERRANT MAST CELL ACTIVITY IN PATIENTS WITH NEUROPROLIFERATIVE VESTIBULODYNIA
Authors: Weinberger J , Goldstein SW , Goldstein I 
Published: December 2024

Introduction

Neuroproliferative vestibulodynia (NPV) is a provoked vestibular pain condition associated with excess immunohistochemical staining for CD117 and PGP9.5, consistent with mast cells and nerves respectively. It is distinguished from control vestibular tissue by having &gt;8 mast cells/high power field. NPV is thus a condition associated with aberrant mast cell activity.

Objective

In patients with histopathologically confirmed NPV, we assessed prevalence of other conditions involving aberrant mast cell activity.

Methods

Medical records from a single facility of patients who underwent a vestibulectomy between June 1, 2019 and May 31, 2024 were reviewed. All patients had histopathologically confirmed NPV. Evidence of other conditions of aberrant mast cell activity was collected based on the medical history form completed by each new patient as well as the history recorded during the initial patient interview, including the following: asthma, chronic sinusitis, endometriosis, food allergy/intolerance, interstitial cystitis (IC), irritable bowel syndrome (IBS), migraine, and postural orthostatic tachycardia syndrome (POTS). That list has been expanded in the last 9 months to include other conditions of aberrant mast cell activity including: seasonal allergies, joint hypermobility, gastroesophageal reflux disease (GERD) and temporomandibular joint (TMJ) dysfunction.

Results

A total of 113 NPV patients (mean age 29, range 19-64 years) were included in this chart review. No other aberrant mast cell activity was reported by 38 (34%), however 75 (66%) had at least one concomitant condition. Of the individuals with other mast cell conditions, the most common were: migraine (n = 29; 26%), IC (n = 25; 22%), endometriosis (n = 20; 18%), food allergy/intolerance (n = 18; 16%), IBS (n = 14; 12%), asthma (n = 9; 8%), chronic sinusitis (n = 5; 6%) and POTS (n = 3; 3%). After assessing for a broader spectrum of conditions of mast cell aberrant activity, additional disorders reported in the last 25 patients with NPV include seasonal allergies (n = 5; 20%), hypermobility (n = 5; 20%); GERD (n = 4; 16%), TMJ (n = 4; 16%). Among this cohort of 113 patients, a total of 40 (35%), 17 (15%), 6 (5%), 5 (4%) and 4 (4%) had one, two, three, four and five or more other conditions involving aberrant mast cell activity, respectively. There was no correlation between the number of concomitant conditions with aberrant mast cell activity reported and the density of CD117-immunopositive staining in the overall NPV cohort.

Conclusions

Patients with histopathologically confirmed NPV have a high prevalence of concomitant conditions associated with aberrant mast cell activity, possibly due to a genetic predisposition. Based on this experience, patients with vulvas are now queried using a more expansive list of conditions of aberrant mast cell activity, as NPV is likely part of a broader systemic collection of these conditions. This finding may be relevant for future diagnostic and treatment options for patients with NPV.

Disclosure

No.