Pyodermatitis Pyostomatitis Vegetans

Abstract

A man in his 20s presented with a 1-month history of extensive and painful vegetating plaques on the lips and buccal and nasal mucosa. His medical history was notable for Crohn disease treated for the previous 6 years with high doses of sulfasalazine (3000 mg/d). A physical examination revealed multiple friable pustules coalescing into vegetativeanderosiveplaqueswithyellowexudateandhemorrhagiccrust on the upper and lower labial mucosa. Similar exophytic plaques were noted to involve the nasal mucosa, resulting in complete obstruction of the nostrils and shortness of breath (Figure). The genital mucosa, intertriginous areas, and scalp were spared. General physical examination results and a review of systems were otherwise normal, and findings from laboratory studies were remarkable for peripheral blood eosinophilia. There was no evidence of active gastrointestinal disease at the time of presentation.A punch biopsy from the lower labial mucosa was performed and revealed epithelial acanthosis, epithelial spongiosis, and intraepithelial neutrophilic and eosinophilic abscesses. Focal suprabasilar clefting and a mixed inflammatory infiltrate within the dermis contained lymphocytes, eosinophils, and neutrophils. There was no evidence of granulomatous inflammation. Results of a direct immunofluorescence study were negative for immunobullous dermatosis. The patient was diagnosed as having a severe and extensive form of pyodermatitis pyostomatitis vegetans (PPV). Treatment with systemic corticosteroids (1 mg/kg/d) was rapidly initiated. Follow-up 1 week later showed a dramatic improvement in the lesions.First described in 1898 by François Hallopeau, PPV is a rare and chronic mucocutaneous dermatosis. Some researchers consider PPV an oral variant of pyoderma gangrenosum, belonging to the spectrum of neutrophilic dermatoses. 1 A strong association with inflammatory bowel diseases is well documented in the literature, particularly with ulcerative colitis. 1,2 Cases associated with primary sclerosing cholangitis have also been reported. Presentations with no underlying systemic disease may be seen. The oral mucosa is the most frequently affected site, but all of the mucosal surfaces can be involved. Clinical presentation is characterized by multiple coalescing pustules on an erythematous base, giving rise to the classic snail

Pyodermatitis-Pyostomatitis Vegetans: A Cutaneous Clue for Crohn’s Disease

“…They are used as systemic, intralesional, and topical treatments depending on disease severity. All the cases reviewed that used corticosteroids achieved clearance, however relapse was common when tapering [1][2][3][4][6][7][8][9][10][11][12][13]. Other systemic treatments for PPV include dapsone, azithromycin,…”

“…Often the clinical courses of PPV and IBD are parallel [1], and successful management of the underlying IBD typically results in resolution of PPV [2]. However, there have also been cases in which PPV develops and persists despite well controlled IBD [1,3,4].…”

“…Pyodermatitis-pyostomatitis vegetans (PPV) is a neutrophilic dermatosis characteristically associated with underlying inflammatory bowel syndrome (IBD) [1]. The first reported case of PPV was in 1898 by Francois Hallopeau [2], and only 37 cases of PPV were reported in the literature between 1979 and 2011 [3]. Diagnosis can be challenging due to its resemblance to autoimmune blistering disorders such as pemphigus vegetans, and infectious etiologies such as Staphylococcus aureus and blastomycosis.…”

Inflammatory bowel disease presenting with pyodermatitis‐pyostomatitis vegetans in a pediatric patient: A case report and review of the literature

“…3 Pyodermatitispyostomatitis vegetans (PD-PSV) is a rare clinical entity manifesting as pustular mucocutaneous vegetative lesions mostly in the oral mucosa, and less commonly affecting the axillary and genital areas. 4 PD-PSV is strongly associated with IBD, most commonly ulcerative colitis and occasionally with CD. 5 Reports on PD-PSV in the pediatric population are scarce.…”

Paediatric Pyodermatitis-Pyostomatitis Vegetans Without Underlying Inflammatory Bowel Disease: A Case Report of a 3-Year-Old African Girl

“…In addition, this condition is often associated with an underlying inflammatory bowel disease (IBD) namely Crohn's disease (CD) and ulcerative colitis (UC). 1,2 There is considerable evidence that the paediatric cases of IBD are on the rise with the peak incidence of IBD occurring in patients between the ages of 15 and 30 years. [1][2][3][4][5] In patients below 20 years of age, CD constitutes nearly 20% of the cases, whereas 12% of them develop UC.…”