Pyodermatitis-pyostomatitis vegetans: a case report and systematic review focusing on oral involvement

Abstract

Background: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare mucocutaneous disease characterized by multiple pustules and it is considered a marker for inflammatory bowel disease (IBD). The oral manifestations of this condition are referred to as pyostomatitis vegetans (PSV).

Purpose: To investigate which features could help in establishing the diagnosis of PSV, with or without cutaneous lesions, based on information retrieved from all cases of PSV described in the literature. A case of PV from the authors was also included in the analysis.

Methods: An electronic search was undertaken, last updated in August 2022. Inclusion criteria included publications reporting cases of PSV, with the diagnosis confirmed by the pathological examination of oral or skin lesions, and presence of IBD.

Results/conclusions: Sixty-two publications with 77 cases of PSV and an associated IBD were included. Features that are helpful in establishing the diagnosis of PSV are snail track appearance of oral lesions, an associated IBD (which is not always symptomatic), evidence of intraepithelial clefting on microscopic examination of oral lesions, and peripheral blood eosinophilia. A gold standard for the management of PSV does not exist and high-level evidence is limited. There is no established therapeutic protocol for PSV and management primarily consists of topical and/or systemic corticosteroids, antirheumatic drugs (sulfasalazine, mesalazine), monoclonal antibody (infliximab, adalimumab) immunosuppressives (azathioprine, methotrexate), antibiotics (dapsone), or a combination of these. The risk of recurrence of oral lesions is considerable when the medication dose is decreased or fully interrupted.

Keywords: Inflammatory bowel disease; Pyodermatitis-pyostomatitis vegetans; Pyostomatitis vegetans; Systematic review.

Fig. 1

Clinical presentation of pyostomatitis vegetans affecting the ventral and lateral borders of the tongue at the initial visit. Notable are exophytic pustular lesions with a “snail track” appearance, erythematous background, and areas of hemorrhage

Fig. 2

“Snail track” lesions involving palatal mucosa

Fig. 3

Pyodermatitis vegetans involving patient’s thigh and manifesting with yellowish crusts surrounded by erythema

Fig. 4

Pyodermatitis vegetans involving the patient’s finger at the initial visit

Fig. 5

Histopathological photomicrograph showing intraepithelial and subepithelial pustules containing neutrophils and eosinophils. H&E, x10

Fig. 6

Histopathological photomicrograph showing acantholysis with intraepithelial clefs formation. H&E, x70