Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rarely described benign inflammatory skin disease of unknown etiology. It is often associated with chronic inflammatory bowel disease. It is common in young and middle-aged individuals, yet rarely in children. Herein, we report the case of a young child who had developed pustular lesions on the skin and mouth as a manifestation of PD-PSV with manifestations associated with primary immune deficiency (PID). Treatment with immunoglobulins and antimycotics resulted in the resolution of the cutaneous and mucosal lesions. To our knowledge, the association between PD-PSV and DIP has not been previously reported. Key words: pyodermatitis, Pyostomatitis, Primary immune deficiency, Pyodermatitis-pyostomatitis vegetans

BackgroundPyodermatitis-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).PurposeTo 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.MethodsAn 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/ConclusionsSixty-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.

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. Some of the lesions assume a 'snail track' appearance. Although there are several similarities between pyodermatitis pyostomatitis vegetans and other diseases, that is pyoderma gangrenosum, pemphigus vegetans and pemphigoid vegetans, the histopathological features of pyodermatitis pyostomatitis vegetans are unique in that epidermal hyperplasia, focal acantholysis and dense inflammatory infiltrates with intraepidermal and subepidermal eosinophilic microabscesses are observed. Direct immunofluorescence findings are principally negative. Activated neutrophils are supposed to play an important role in the pathogenesis of pyodermatitis pyostomatitis vegetans. The expression of IL-36 and neutrophil extracellular traps (NETs) was observed in the lesional skin, and additionally, eosinophil extracellular traps (EETs) was detected in pyodermatitis pyostomatitis vegetans. A possible pathogenic role of NETs and EETs in the innate immunity and autoinflammatory aspects of pyodermatitis pyostomatitis vegetans was discussed.

Dermatologic Manifestations of Systemic Diseases in Childhood

Background: Pyodermatitis-pyostomatitis vegetans (PDV-PSV) is a rare muco-cutaneous disorder characterized by vegetating and pustular plaques and is often associated with inflammatory bowel disease (IBD). The purpose of this study was to systematically identify and analyze reports of PDV-PSV to determine the most effective treatment. Methods: Reports of PDV-PSV were identified using the OVID-Medline database from inception through November 2019. Publications were excluded if no new patient case was included, if there was not clinical and histological evidence of PDV, PSV, or PDV-PSV, or if no treatment was discussed. Results: The final sample was comprised of 74 publications plus an additional patient from the authors’ institution, corresponding to 95 total patients. The basis of the review and analysis is limited to case reports and case series, which likely only report the cases with positive outcomes. Statistical analysis revealed that oral corticosteroids (OCS), 6-mercaptopurine/azathioprine, oral calcineurin inhibitors (OCNI), 5-aminosalicylic-acid (5-ASA), and biologics (BIO) were the most effective treatments for PDV-PSV. Topical medications, colchicine, oral dapsone, and other antibiotics were ineffective treatments, with topical medications being the least effective option. When OCS are used, they work best when used as initial treatment to induce remission. 5-ASA and BIO are most effective when used as maintenance therapies after initial remission. Conclusions: Thus, first line therapy for PDV-PSV should begin with OCS with transition to steroid-sparing agents including OCNI, BIO, and 5-ASA if indicated.

Pyoderma gangrenosum (PG) belongs to the group of neutrophilic dermatoses with unknown etiology and poorly understood pathogenesis. In children, PG is primarily associated with inflammatory bowel diseases (Crohn's disease and ulcerative colitis). By now, about 355 PG cases have been described worldwide, including 15 children with the involvement of oral mucosa. Clinical manifestations of the disease are diverse and depend on the form, stage and associated conditions. Such a rare PG as pyodermatitis-pyostomatitis vegetans manifests by combined lesions of the oral mucosa and skin. The authors present historical data on the investigation of the disease, its etiology, pathogenesis, risk factors, and clinical picture. A description of a rare clinical case of severe form of atypical PG, namely, pyodermatitis-pyostomatitis vegetans in a 10-year-old child, is presented. The unique character of the case is related to the variety of clinical manifestations and the clinical course complicated by the development of keloid and partial scar-related flexion contracture of the upper and lower extremities, the formation of microstoma and cachexia. The association of pyodermatitis-pyostomatitis vegetans with Crohn's disease was found. The lack of awareness of the clinical symptoms of this rare disease has led to diagnostic errors and late diagnosis.

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. The total number of cases was 38, including 22 men and 16 women, with an average age of forty. Direct immunofluorescence staining had been performed for 32 patients, of which 12 had positive findings. Of those with positive direct immunofluorescence, 6 patients showed IgA cell surface staining. A recent approach suggests that these immunological findings may not be accidental and indicates a possible overlap with autoimmune bullous diseases discussed in this review.

Low-dose sulfasalazine in a case of pyodermatitis-pyostomatitis vegetans

Pyodermatitis-pyostomatitis vegetans (PD-PSV)), is a very rare, chronic inflammatory dermatosis. It is characterized by large exudative, verrucous plaques with well-defined and elevated borders with typical mucocutaneous involvement.

A 58-year-old female was admitted to the hospital for a 3-month history of erythema,pustules and vegetating plaques on the lips and scalp,as well as in intertriginous areas.She had a 2-year history of ulcerative colitis,which had been aggravated before the development of skin lesions.Skin examination demonstrated diffuse thickening of both lips covered with multiple granule-to mung bean-sized confluent pustules.Yellow crusts were observed at the lip margins.A 1.5 cm × 2 cm vegetating plaque surrounded by anular pustules was found in the left buccal mucosa.There were multiple irregularly-sized vegetating plaques with erosions and yellow crusts on the scalp,neck,as well as in the periumbilical and left inguinal and axillary region,and annular vesicles and pustules were observed in the center and margin of these plaques.The lesions subsided leaving hyperpigmented macules.There were four fresh pustules in the right axillary region.Histological examination of a biopsy specimen taken from the lower lip margin revealed mild hyperplasia of and neutrophil microabscess in the epidermis,as well as a dense inflammatory infiltrate composed mostly of neutrophils and eosinophils with microabscess formation in the dermis.Direct immunofluorescence examination of the normal-appearing skin next to the lesions was negative.The percentage of peripheral blood eosinophil was slightly elevated.Bacterial and fungal cultures of pustular contents were negative.A diagnosis of pyodermatitis-pyostomatitis vegetans was made. Key words: Pyodermatitis-pyostomatitis vegetans

Pyodermatitis-pyostomatitis vegetans (PPV) constitutes an inflammatory mucocutaneous dermatosis that is associated with inflammatory bowel disease. Clinically, PPV appears as pustules on mucosal surfaces and as vegetating exudative plaques on intertriginous surfaces. It is typically a clinical diagnosis supported by histological findings. Microscopic findings include epidermal hyperplasia, focal acantholysis, and a dense mixed inflammatory infiltrate with intraepithelial and subepithelial eosinophilic microabscesses. In the recent literature, immunofluorescence has been thought to be negative in PPV or, if positive, an aberrant finding. Herein, we report 2 cases of PPV associated with inflammatory bowel disease, which display intercellular IgA deposits. Although these cases may represent isolated epiphenomena, it is possible that the paucity of PPV cases with immunofluorescent studies hitherto has led to an oversight of an interesting association between intercellular IgA and PPV.

Pyodermatitis-pyostomatitis vegetans: a differential diagnosis of pemphigus vegetans

Pyodermatitis-pyostomatitis vegetans is a rare inflammatory dermatosis of unknown etiology, with a typical mucocutaneous involvement. We report the case of a woman with pustular and vesicular lesions in the axillae, evolving with vegetating plaques and pustules with annular grouping. The disease progressed with vulvar and inguinal involvement as well as involvement of the oral, nasal and ocular mucous membranes. She started the treatment with prednisone (40 mg/day), with remission of the lesions after one month of use of such medication. Association with inflammatory bowel disease occurs in 70% of the cases. Immunofluorescence, which is typically negative, helps to characterize the disease. A rapid response to systemic steroids is expected.

Clinical & Experimental OphthalmologyVolume 39, Issue 9 p. 910-911 Eyelid deformities from pyodermatitis pyostomatitis vegetans Gregory Moloney FRANZCO, Gregory Moloney FRANZCO University of British Columbia Department of Visual Sciences, Oculoplastic Unit, Section I, Eye Care Centre, Vancouver, British Columbia, CanadaSearch for more papers by this authorPeter J Dolman FRCSC, Peter J Dolman FRCSC University of British Columbia Department of Visual Sciences, Oculoplastic Unit, Section I, Eye Care Centre, Vancouver, British Columbia, CanadaSearch for more papers by this author Gregory Moloney FRANZCO, Gregory Moloney FRANZCO University of British Columbia Department of Visual Sciences, Oculoplastic Unit, Section I, Eye Care Centre, Vancouver, British Columbia, CanadaSearch for more papers by this authorPeter J Dolman FRCSC, Peter J Dolman FRCSC University of British Columbia Department of Visual Sciences, Oculoplastic Unit, Section I, Eye Care Centre, Vancouver, British Columbia, CanadaSearch for more papers by this author First published: 18 April 2011 https://doi.org/10.1111/j.1442-9071.2011.02583.xCitations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume39, Issue9December 2011Pages 910-911 RelatedInformation

Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis often associated with gastrointestinal disorders, especially with inflammatory bowel disease. It is clinically characterized by erythematous lesions with multiple pustules and erosions affecting the mucosal surfaces. Cutaneous lesions are characterized by exudative and vegetating plaques affecting frequently the axillae and groins. The clinical diagnosis is supported by histologic findings, whereas immunofluorescence studies are useful to rule out other entities such as pemphigus3. Herein we report the case of a young man who was misdiagnosed as having IgA pemphigus for 8 years due to positive immunofluorescence findings. The clue for the final diagnosis was the diagnosis of a concomitant ulcerative colitis, which prompted us to reconsider his cutaneous disease.

A 51-year-old Japanese woman underwent subtotal colectomy for ulcerative colitis 5 years before her first visit to our hospital, and her symptoms stabilized. She presented with a 2-month history of proliferative, pustular lesions on her face, scalp, and gingival and anal mucosae. Histological examination showed features similar to those of pemphigus vegetans. However, the results of immunofluorescence studies, immunoblot analysis and enzyme-linked immunosorbent assay for pemphigus were negative, and colonoscopy revealed a relapse of ulcerative colitis in the remaining rectal mucosa, so a diagnosis of pyodermatitis-pyostomatitis vegetans was made. This is the first report of mucocutaneous lesions that developed with a relapse of ulcerative colitis in the remaining rectum after subtotal colectomy. Moreover, a close correlation was found between the condition of bowel inflammation and that of mucocutaneous lesions.

Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic pustular and vegetating mucocutaneous dermatosis. The oral lesions present as multiple, friable and yellowish pustules, which form "snail tracts" and rupture easily. The cutaneous lesions begin as crusted erythematous papulopustules that coalesce to form large vegetating plaques, usually in the axillae, genital area and scalp. Cutaneous lesions usually develop at the same time as the oral lesions or thereafter. PPV is usually considered as a specific marker for inflammatory bowel disease since the concurrence of PPV and inflammatory bowel disease has been reported in approximately 70% of cases. We report two patients who showed typical clinical and histopathological features of PPV, but were not accompanied by inflammatory bowel disease.

Pyostomatite-pyodermatite végétante avec atteinte nasale

Pyodermatitis–Pyostomatitis Vegetans of the Eyelids: Case Report and Review of the Literature

Pyodermatitis-pyostomatitis vegetans (PPV), a rare disorder of the skin and oral mucosa, is considered a highly specific marker for inflammatory bowel disease, especially ulcerative colitis (UC). Oral lesions (pyostomatitis vegetans) are seen without skin involvement but rarely without gastrointestinal symptoms. Bowel symptoms may be minimal and precede the onset of other lesions by months or years. Dermatologically, PPV is characterized by annular, pustular lesions, which may precede or appear at the same time as the oral lesions. We report a case of PPV and UC in which presentation was confused by acneiform lesions and methicillin-resistant Staphylococcus aureus colonization. Management was complicated because of the patient's job commitments and need to travel, and the involvement of a number of different specialties at different locations.