Pyostomatitis vegetans

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...

Neutrophilic dermatoses are a broadly heterogeneous group of inflammatory skin disorders. This article reviews 5 conditions: amicrobial pustulosis of the folds, aseptic abscess syndrome, Behçet disease, neutrophilic eccrine hidradenitis, and pyostomatitis vegetans-pyodermatitis vegetans.The authors include up-to-date information about their epidemiology, pathogenesis, clinicopathologic features, diagnosis, and management.

A man in his early 50s was referred to the oral medicine clinic regarding a 6-month history of ulceration affecting the palate and gingivae. Intra-oral examination revealed multiple friable pustules on a background of erythema affecting the gingival sulci and 'snail-track' like ulceration affecting the palate. Histopathological analysis of the gingivae revealed features in keeping with a diagnosis of pyostomatitis vegetans (PV). Although he did not report any gastrointestinal symptoms, the patient was referred to gastroenterology and underwent colonoscopy and small bowel series examination...

Pyodermatitis pyostomatitis vegetans is a rare inflammatory condition, affecting the skin and/or mucous membrane. Some cases include both skin and mucous involvement, whereas others develop either skin or mucous lesions only. The typically affected areas are the scalp, face, trunk and extremities, including the flexural areas and umbilicus. Clinical features show erosive granulomatous plaques, keratotic plaques with overlying crusts and pustular lesions. Among mucous lesions, oral mucosa is most frequently involved, and gingival erythema, shallow erosions, cobblestone-like papules on the buccal mucosa or upper hard palate of the oral cavity are also observed...

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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...

Pyostomatitis vegetans (PSV) is a rare disorder strongly associated with inflammatory bowel disease (IBD), most commonly ulcerative colitis, but has infrequently been found in patients with Crohn disease (CD). We present the case of a 7-year-old girl with PSV and extraintestinal findings suggestive of CD-chelitis granulomatosa, perianal and vulvar edema with biopsies revealing noncaseating granulomas, and anal skin tags-and an elevated calprotectin noted during a cutaneous flare. She did not have clinical or endoscopically identified underlying gastrointestinal involvement for 4 years after symptom onset...

Pyodermatitis-Pyostomatitis Vegetans (PD-PSV) remains a rare disorder of unknown etiology that is often associated with underlying inflammatory bowel disease (IBD). It is also worth noting that PD-PSV is particularly rare in the paediatric population with 15.4 years as the average age of onset in reported cases. Although adults and children with IBD may exhibit similar clinical features, children often manifest with additional features of failure to thrive and delayed puberty due to the background chronic inflammatory process...

As an immune dysregulation-related disease, although ulcerative colitis (UC) primarily affects the intestinal tract, extraintestinal manifestations of the disease are evident, particularly in the oral cavity. Herein, we have reviewed the various oral presentations, potential pathogenesis, and treatment of oral lesions related to UC. The oral manifestations of UC include specific and nonspecific manifestations, with the former including pyostomatitis vegetans and the latter encompassing recurrent aphthous ulcers, atrophic glossitis, burning mouth syndrome, angular cheilitis, dry mouth, taste change, halitosis, and periodontitis...

Inflammatory bowel diseases (IBD) are chronic inflammatory disorders of the digestive tract, and they involve systemic inflammatory diseases known as extra-intestinal manifestations (EIMs). Timely and correct diagnosis of mucocutaneous EIMs could assist with detecting and monitoring IBD. We present a case of 52-year-old male patient of ulcerative colitis with 2 rare EMIs together at the same time: pyostomatitis vegetans in the oral cavity and Sweet syndrome on the skin. They presented as multiple small white or yellow pustules on the surface of the hyperemic fragile oral mucosa and abrupt appearance of painful, swollen, and erythematous papules on the skin, respectively...

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New England Journal of Medicine, Volume 385, Issue 22, November 2021.

No abstract text is available yet for this article.

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is rarely reported in the pediatric population. Here, we provide a review of pediatric PD-PSV in the literature and report a case of widespread PD-PSV in a 15-year-old male without a previous history of inflammatory bowel disease or gastrointestinal symptoms. Clinical, histological, and immunopathological workup established PD-PSV and revealed subclinical Crohn's disease. Treatment with infliximab was effective in inducing rapid resolution of the lesions.

A 51-year-old woman presented with a 4-month history of painful ulcers in the mouth and vulva, and painful vegetative plaques at intertriginous sites. Skin biopsies showed squamous hyperplasia and intraepidermal eosinophilic pustulation. Skin direct immunofluorescence (DIF) revealed intercellular deposition of IgG and C3 in the lower part of the epidermis, while serum indirect immunofluorescence (IIF) confirmed the presence of antiepithelial antibodies. The patient was diagnosed with pemphigus vegetans, and successfully treated with dapsone, prednisolone and topical steroids...

Inflammatory bowel diseases (IBDs) may be associated with extra-intestinal manifestations. Among these, mucocutaneous manifestations are relatively frequent, often difficult to diagnose and treat, and may complicate the course of the underlying disease. In the present review, a summary of the most relevant literature on the dermatologic manifestations occurring in patients with inflammatory bowel diseases has been reviewed. The following dermatological manifestations associated with IBDs have been identified: (i) specific manifestations with the same histological features of the underlying IBD (occurring only in Crohn's disease); (ii) cutaneous disorders associated with IBDs (such as aphthous stomatitis, erythema nodosum, psoriasis, epidermolysis bullosa acquisita); (iii) reactive mucocutaneous manifestations of IBDs (such as pyoderma gangrenosum, Sweet's syndrome, bowel-associated dermatosis-arthritis syndrome, aseptic abscess ulcers, pyodermatitis-pyostomatitis vegetans, etc...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

UNLABELLED: Background and Objectives : Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime...

Pyodermatitis-pyostomatitis vegetans is a rare inflammatory dermatosis. There is a strong association between pyodermatitis-pyostomatitis vegetans and inflammatory bowel disease, particularly ulcerative colitis. Herein, we report a case of pyodermatitis-pyostomatitis vegetans with positive direct immunofluorescence staining findings and review the literature for the past 18 years to characterize the disease, its epidemiologic characteristics, its associations, and the pathology and direct and indirect immunofluorescence findings...