RESUMO
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. A case of PV from the authors was also included in the analysis. METHODS: An 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/CONCLUSIONS: Sixty-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.
Assuntos
Pioderma , Estomatite , Humanos , Estomatite/tratamento farmacológico , Estomatite/patologia , Estomatite/diagnóstico , Pioderma/tratamento farmacológico , Pioderma/patologia , Pioderma/diagnóstico , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/tratamento farmacológico , Feminino , MasculinoRESUMO
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.
Assuntos
Armadilhas Extracelulares , Pênfigo , Pioderma , Estomatite , Humanos , Pioderma/complicações , Pioderma/patologia , Estomatite/etiologia , Estomatite/patologia , Neutrófilos/patologia , Eritema , Compostos OrgânicosRESUMO
Blastomycosis-like pyoderma (BLP) is an uncommon tissue response possibly to bacterial infection that presents as vegetative skin lesions usually in immunocompromised patients. Staphylococcus aureus is the most frequent pathogen implicated in BLP. Here, we report the case of a 32-year-old man who had ulcerative vegetating lesions on extremities for 3 months over preexisting recalcitrant tinea lesions. The patient was hypertensive with a history of chronic graft rejection after renal transplantation 8 months earlier and was on long-term immunosuppressants. Investigations revealed anemia, mild hyperglycemia, and elevated serum creatinine. Histopathology showed suppurative and fibrosing perifolliculitis with moderate pseudocarcinomatous hyperplasia and the culture of biopsy specimen demonstrated growth of Escherichia coli and Citrobacter koseri. The diagnosis of BLP overlying dermatophytoses was made. The skin lesions improved completely with parenteral antibiotics. Local immune dysregulation by dermatophytoses along with iatrogenic immunosuppression may have favored this rare pyoderma.
Assuntos
Pioderma , Tinha , Masculino , Humanos , Adulto , Pioderma/diagnóstico , Pioderma/microbiologia , Pioderma/patologia , Antibacterianos/uso terapêutico , Imunossupressores , Hospedeiro Imunocomprometido , Tinha/diagnóstico , Tinha/tratamento farmacológico , Tinha/microbiologiaRESUMO
Blastomycosis-like pyoderma is a rare cutaneous disease presenting as solitary or multiple verrucous or ulcerated plaques and nodules in a susceptible patient. The diagnostic criteria include characteristic verrucous plaques with pustules and elevated borders, histopathologic findings of pseudoepitheliomatous hyperplasia with abscesses, growth of at least one bacterium in tissue culture, and exclusion of other infectious sources. This report describes a case of a 62-year-old man with poorly controlled type 2 diabetes mellitus who presented with plaques, nodules, and ulcers in both groins and the right ankle. The patient was initially misdiagnosed with multiple squamous cell carcinomas and underwent several operations. A review of the pathology slides revealed pseudoepitheliomatous hyperplasia with multiple dermal abscesses, while repeated wound and tissue cultures were positive for coagulase-negative Staphylococcus. Blastomycosis-like pyoderma was diagnosed. The patient was subsequently treated with culture-guided prolonged antibiotic therapy followed by intralesional steroid injection, which led to gradual resolution of the lesions.
Assuntos
Blastomicose , Carcinoma de Células Escamosas , Diabetes Mellitus Tipo 2 , Pioderma , Dermatopatias , Masculino , Humanos , Pessoa de Meia-Idade , Blastomicose/diagnóstico , Hiperplasia , Abscesso/diagnóstico , Pioderma/diagnóstico , Pioderma/patologia , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patologia , Dermatopatias/diagnóstico , Erros de Diagnóstico , Diagnóstico DiferencialRESUMO
Background: The Burkholderia cepacia complex (Bcc) is an opportunistic pathogen in humans and animals. Deep pyoderma caused by these bacteria in dogs has been previously reported. This case series aims to describe contrasting treatment responses in Bcc-related deep pyoderma in two dogs, a male and a female. Case Description: Both patients had a history of immune-mediated polyarthritis (IMPA) managed with oral ciclosporin and prednisolone. Their skin lesions were multifocal, irregular, erythematous to hemorrhagic, alopecic papules, plaques, and nodules, with extensive crusting, draining tracts, and ulceration. Cytological findings revealed a marked inflammatory response consisting of non-degenerative and degenerative neutrophils and macrophages, with moderate to abundant intracellular and extracellular Bcc. Ciclosporin and prednisolone were stopped in case 2 after diagnosis. However, it was challenging to stop the regimen in case 1 because of the recurrence of IMPA and the onset of iatrogenic hypoadrenocorticism. Case 1 did not achieve remission for approximately 66 weeks even with seven protocols because of multiple relapses, whereas it took only 3 weeks to achieve remission in case 2 while using one drug. Conclusion: For deep pyoderma with extensive lesions in immunosuppressed patients, one should consider infection with Bcc. Therefore, immunosuppressants should promptly be reduced in such patients, and then, intensive antimicrobial therapy may achieve remission.
Assuntos
Complexo Burkholderia cepacia , Doenças do Cão , Pioderma , Animais , Antibacterianos/uso terapêutico , Ciclosporina/uso terapêutico , Doenças do Cão/patologia , Cães , Feminino , Humanos , Masculino , Prednisolona/uso terapêutico , Pioderma/tratamento farmacológico , Pioderma/patologia , Pioderma/veterináriaRESUMO
We present two cases of vegetating exudative lesions involving the oral mucosa, in patients that are cocaine users, with findings in biopsy and in direct immunofluorescence consistent with the diagnosis of pyostomatitis vegetans-pyodermatitis vegetans.
Assuntos
Cocaína , Pênfigo , Pioderma , Estomatite , Cocaína/efeitos adversos , Humanos , Mucosa Bucal/patologia , Pênfigo/patologia , Pioderma/patologia , Estomatite/patologiaAssuntos
Blefarite/microbiologia , Pioderma/patologia , Dermatopatias Bacterianas/patologia , Estomatite/patologia , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Antivirais/uso terapêutico , Blefarite/diagnóstico , Blefarite/etiologia , Blefarite/patologia , Doença de Crohn/complicações , Diagnóstico Diferencial , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Herpes Simples/genética , Humanos , Pessoa de Meia-Idade , Mucosa Bucal/patologia , Mucosa Bucal/virologia , Pioderma/microbiologia , Salmonella/genética , Salmonella/isolamento & purificação , Dermatopatias Bacterianas/complicações , Dermatopatias Bacterianas/microbiologia , Dermatopatias Bacterianas/virologia , Estomatite/diagnóstico , Estomatite/virologia , Resultado do TratamentoAssuntos
Granuloma/patologia , Imunoglobulina A/sangue , Mieloma Múltiplo/patologia , Pioderma/patologia , Dermatopatias Vesiculobolhosas/patologia , Assistência ao Convalescente , Idoso , Anti-Infecciosos/administração & dosagem , Anti-Infecciosos/uso terapêutico , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Biópsia/métodos , Medula Óssea/patologia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dapsona/administração & dosagem , Dapsona/uso terapêutico , Quimioterapia Combinada , Granuloma/complicações , Humanos , Hiperpigmentação/patologia , Masculino , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico , Dermatopatias Vesiculobolhosas/complicações , Dermatopatias Vesiculobolhosas/diagnóstico , Resultado do TratamentoRESUMO
Superficial granulomatous pyoderma (SGP) is a rare pyoderma gangrenosum (PG) variant that differs from classic PG in that the ulcers tend to be more superficial, lack a rapidly advancing border, and are not typically associated with an underlying systemic disease. The ulcers are most commonly painless and located on the trunk, with a clean granulating base. They generally do not show undermining but may have a vegetative border. Lesions usually respond well to either topical or intralesional corticosteroids with complete healing. The classic histopathologic finding is a "three-layer granuloma" in the superficial dermis consisting of central neutrophilic inflammation and necrosis, a surrounding layer of histiocytes and multinucleated giant cells, and an outer most layer of plasma cells and eosinophils. Herein, we present a unique case of SGP with sporotrichoid-like distribution on the lower extremity.
Assuntos
Granuloma/patologia , Pioderma/patologia , Administração Tópica , Corticosteroides/administração & dosagem , Idoso , Diagnóstico Diferencial , Granuloma/diagnóstico , Granuloma/tratamento farmacológico , Humanos , Extremidade Inferior/patologia , Masculino , Pioderma/diagnóstico , Pioderma/tratamento farmacológicoRESUMO
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.
Assuntos
Mucosa Bucal/patologia , Pioderma/patologia , Estomatite/patologia , Adulto , Doenças Autoimunes/imunologia , Feminino , Técnica Direta de Fluorescência para Anticorpo , Humanos , Imunoglobulina A/análise , Masculino , Pioderma/imunologia , Dermatopatias Vesiculobolhosas/imunologia , Estomatite/imunologiaAssuntos
Doenças Autoimunes/tratamento farmacológico , Vesícula/tratamento farmacológico , Fármacos Dermatológicos/uso terapêutico , Infliximab/uso terapêutico , Pioderma/tratamento farmacológico , Estomatite/tratamento farmacológico , Doenças Autoimunes/patologia , Vesícula/patologia , Diagnóstico Tardio , Feminino , Humanos , Pessoa de Meia-Idade , Pioderma/patologia , Estomatite/patologia , Resultado do TratamentoRESUMO
Necrobiosis lipoidica (NL) is a granulomatous inflammatory skin disease strongly associated with diabetes mellitus (DM). Red-brown papules expanding into plaques with erythematous indurated borders on the lower extremities are characteristic of NL. Diagnosis is made clinically; however, biopsy of lesions confirms the diagnosis. Untreated NL may ulcerate and lead to further complications, but progression to superimposed pyoderma vegetans (PV) is not a known occurrence.
Assuntos
Necrobiose Lipoídica/patologia , Pioderma/patologia , Adulto , Antibacterianos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Betametasona/análogos & derivados , Betametasona/uso terapêutico , Cefalexina/uso terapêutico , Feminino , Humanos , Mupirocina/uso terapêutico , Necrobiose Lipoídica/terapia , Pioderma/terapia , Dermatopatias Bacterianas/patologia , Dermatopatias Bacterianas/terapiaAssuntos
Acantólise/diagnóstico , Imunoglobulina A/imunologia , Pioderma/diagnóstico , Estomatite/diagnóstico , Acantólise/patologia , Biópsia , Diagnóstico Diferencial , Humanos , Masculino , Pênfigo/diagnóstico , Pênfigo/patologia , Pênis/imunologia , Pênis/patologia , Pioderma/patologia , Pele/imunologia , Pele/patologia , Estomatite/patologiaAssuntos
Endocardite Bacteriana/microbiologia , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Infecções Relacionadas à Prótese/microbiologia , Pioderma/microbiologia , Dermatoses do Couro Cabeludo/microbiologia , Alopecia/terapia , Antibacterianos/uso terapêutico , Biópsia , Ecocardiografia , Endocardite Bacteriana/sangue , Endocardite Bacteriana/diagnóstico por imagem , Endocardite Bacteriana/tratamento farmacológico , Glucocorticoides/uso terapêutico , Cabelo , Humanos , Masculino , Próteses e Implantes/efeitos adversos , Implantação de Prótese/efeitos adversos , Infecções Relacionadas à Prótese/complicações , Infecções Relacionadas à Prótese/tratamento farmacológico , Infecções Relacionadas à Prótese/patologia , Pioderma/complicações , Pioderma/tratamento farmacológico , Pioderma/patologia , Dermatoses do Couro Cabeludo/complicações , Dermatoses do Couro Cabeludo/tratamento farmacológico , Dermatoses do Couro Cabeludo/patologia , Pele/patologiaAssuntos
Pustulose Exantematosa Aguda Generalizada/diagnóstico , Pustulose Exantematosa Aguda Generalizada/tratamento farmacológico , Pioderma/diagnóstico , Pioderma/tratamento farmacológico , Adolescente , Biópsia por Agulha , Dapsona/uso terapêutico , Diagnóstico Diferencial , Quimioterapia Combinada , Floxacilina/uso terapêutico , Humanos , Imuno-Histoquímica , Quênia , Masculino , Prednisolona/uso terapêutico , Pioderma/patologia , Doenças Raras , Medição de Risco , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
BACKGROUND: Lympho-epithelial Kazal-type inhibitor (LEKTI) tightly controls the activities of serine proteases such as kallikrein-related peptidase (KLK) 5 and KLK7 in the epidermis. LEKTI is known to be an essential molecule for the epidermal skin barrier, as demonstrated by SPINK5 nonsense mutation, which results in Netherton syndrome. Toll-like receptors (TLRs) recognize pathogen-associated molecular patterns or damage-associated molecular patterns and produce inflammatory cytokines, chemokines, and antimicrobial peptides. However, the effect of TLR signaling on the expression of LEKTI is not clear. OBJECTIVE: To investigate whether TLR signaling can affect expression of LEKTI in epidermal keratinocytes. METHODS: We stimulated a panel of TLR ligands and investigated the expression of LEKTI in normal human epidermal keratinocytes (NHEKs). We further measured trypsin or chymotrypsin-like serine protease activity in NHEK cultured media under stimulation with TLR3 ligand, poly (I:C). Immunostaining for LEKTI was performed using skin samples from skin infectious diseases. RESULTS: TLR1/2, 3, 5, and 2/6 ligands induced the expression of LEKTI in NHEKs. The trypsin or chymotrypsin-like serine protease activity in NHEKs was up-regulated with the stimulation of poly (I:C). The gene expressions of KLK6, KLK10, KLK11, and KLK13 were also increased by poly (I:C). An immunohistochemical analysis demonstrated that the expression of LEKTI was up-regulated in the lesions of varicella, pyoderma, and rosacea. CONCLUSIONS: TLR signaling induces the expression of LEKTI in epidermal keratinocytes, which might contribute to the control of aberrant serine protease activities in inflammatory skin diseases.