(048) PREVALENCE OF CONDITIONS INVOLVING ABERRANT MAST CELL ACTIVITY MAY HELP IDENTIFY PATIENTS WITH NEUROPROLIFERATIVE VESTIBULODYNIA
Authors: Goldstein SW , Goldstein I , Kim NN 
Published: December 2024

Introduction

Neuroproliferative vestibulodynia (NPV) is a provoked genital pain characterized by severe allodynia, hyperalgesia and entry dyspareunia. Bornstein et al. determined the parameters for diagnostic confirmation of NPV to be &gt;8 cells positively immunostained for CD117 (mast cell marker) in a field viewed at 100× magnification. Since the cellular pathology of NPV is not visible during physical examination and lies underneath the vestibular epithelium, many patients with NPV have been examined by multiple clinicians and have undergone numerous unsuccessful diagnostic tests and treatments. We noted that patients with documented NPV by histomorphometric quantification of mast cells reported other concomitant medical conditions associated with aberrant mast cell activity. Thus, taking a detailed history of patients with suspected NPV for the presence of other mast cell related conditions may assist in the identification of NPV.

Objective

In patients with a histopathologically confirmed diagnosis of NPV, we wished to assess the prevalence of concomitant aberrant mast cell activity conditions.

Methods

An IRB exempt chart review of people with vulvas who underwent vestibulectomy between June 1, 2019 and October 31, 2023 was performed at our facility. Histories from these patients, all of whom had histopathologically confirmed NPV after observing &gt;8 positive CD117 stained cells/high power field in the sub-epithelial stroma, were reviewed. Conditions of aberrant mast cell activity reported by patients as part of their history was noted. Focus was on the following eight conditions: asthma, chronic sinusitis, endometriosis, food allergy/intolerance, interstitial cystitis, irritable bowel syndrome, migraine, and postural orthostatic tachycardia syndrome.

Results

A total of 88 NPV patients were included in this chart review. While 34 (39%) reported no other aberrant mast cell activity, 54 (51%) had at least one concomitant condition. A total of 33 (38%), 13 (15%), 3 (6%), 1 (1%) and 2 (2%) had one, two, three, four and five other conditions involving aberrant mast cell activity, respectively. The most common conditions were migraine (n = 21; 24%), interstitial cystitis (n = 17; 19%) and endometriosis (n = 15; 17%), followed by food allergy/intolerance (n = 12; 14%), irritable bowel syndrome (n = 9; 10%), asthma (n = 9; 10%), chronic sinusitis (n = 5; 6%) and postural orthostatic tachycardia syndrome (n = 2; 2%). As these data are based on self-report during patient intake, and not on a complete assessment of all aberrant mast cell activity conditions, actual prevalence may be higher. 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 or within the subgroups with either lifelong or acquired NPV.

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, making them more susceptible to mast cell conditions. While more studies are needed, NPV is likely part of a broader systemic collection of conditions associated with aberrant mast cell activity and may serve to help diagnose and direct treatment of NPV in the future.

Disclosure

No.