# Symptomatic Vulvar Primary Cutaneous Amyloidosis Associated With Low–Risk HPV: A Case Report

## TL;DR

A rare case of vulvar amyloidosis linked to low-risk HPV is reported, emphasizing the need for accurate diagnosis and the possible role of HPV in amyloid formation.

## Contribution

This case report is the first to associate low-risk HPV with vulvar primary cutaneous amyloidosis, suggesting a potential etiological link.

## Key findings

- A 73-year-old woman with a vulvar lesion was diagnosed with primary localized cutaneous amyloidosis.
- HPV type 40, a low-risk genotype, was detected without evidence of intraepithelial neoplasia.
- The patient remained in remission after surgical excision with no recurrence after 33 months.

## Abstract

Primary localized cutaneous amyloidosis (PCLA) of the vulva is an infrequent diagnosis. Its clinical presentation may mimic neoplastic or inflammatory lesions and could even be associated with human papillomavirus (HPV) of low or high oncogenic risk, making accurate diagnosis and exclusion of systemic involvement essential. A 73‐year‐old woman presented with a flat, macular, solitary vulvar lesion. Histologic evaluation revealed amyloid deposits in the papillary dermis, confirmed by Congo red staining and apple‐green birefringence. HPV genotyping identified type 40, a low‐risk genotype, with no evidence of intraepithelial neoplasia. The diagnosis of systemic amyloidosis was ruled out by a multidisciplinary team evaluation. A complete local excision was performed. After 33 months of follow‐up, the patient remains in remission. This case highlights the importance of including PCLA in the differential diagnosis of vulvar lesions and suggests a potential role of HPV as a possible trigger for vulvar localized amyloidogenesis.

Primary vulvar cutaneous amyloidosis is a rare non‐neoplastic lesion that should be considered in differential diagnoses, with HPV infection potentially contributing to amyloid deposition even without VIN. Surgical resection may be an alternative in select cases, though systemic amyloidosis must first be excluded.