Case of Pyodermatitis Pyostomatitis Vegetans- JAMA Dermatolo
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PPV an oral variant of pyoderma gangrenosum, belonging to the spectrum of neutrophilic dermatoses.A strong association with inflammatory bowel diseases is well documented in the literature, particularly with ulcerative colitis.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 tracks erosions.

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 vegetative and erosive plaques with yellow exudate and hemorrhagic crust 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.

Conclusively, It is important to perform a thorough gastrointestinal assessment when confirming PPV so that subclinical inflammatory bowel disease can be diagnosed early. Systemic corticosteroids are the first-line treatment. However, the condition is likely to relapse during tapering withdrawal of medication. As a second-line therapy, treatment options such as dapsone, azathioprine, cyclosporine, methotrexate, and infliximab have been reported with varying results. Treatment of the associated inflammatory bowel disease often results in an improvement of the mucocutaneous lesions.

Source: https://jamanetwork.com/journals/jamadermatology/article-abstract/2757504
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