Idiopathic pyostomatitis-pyodermatitis vegetans with nasal obstruction: A case report

Abstract

Pyostomatitis-pyodermatitis vegetans is an uncommon mucocutaneous dermatosis of unknown etiology. It presents as erythematous pustules that coalesce to form exudative vegetating plaques on the oral mucosa and/or skin. Diagnosis is largely based on clinical assessment, although histopathology should be used as supporting evidence. Pyostomatitis-pyodermatitis vegetans is associated with inflammatory bowel disease, liver dysfunction, and others. We report a case of a 48-year-old man who presented with erythematous eroded plaques involving his nares and upper lip. Investigations revealed a dense mixed inflammatory infiltrate and pronounced peripheral eosinophilia. Uniquely, the patient did not have inflammatory bowel disease or any other systemic condition. Systemic corticosteroids were helpful in clearing mucocutaneous lesions; however, this effect was only sustained at high doses. There are few reports of pyostomatitis-pyodermatitis vegetans in the medical literature. This case highlights key aspects of this extremely rare dermatosis, especially in otherwise healthy patients without inflammatory bowel disease.

Keywords: Pyostomatitis-pyodermatitis vegetans; inflammatory bowel disease; mucocutaneous dermatosis; peripheral blood eosinophilia.

Figure 1.

(a) Coalescing eroded plaques with a snail track appearance obstructing anterior nares. (b) Presentation 3 weeks after initiating treatment with systemic corticosteroids.

Figure 2.

(a) Low-power H + E micrograph depicting a dense infiltrate of plasma cells and eosinophils with destruction of epidermis and appendageal structures. (b) High-power H + E micrograph showing dense eosinophilic infiltrate without cellular atypia.