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Abscesso , Pioderma Gangrenoso , Humanos , Pioderma Gangrenoso/diagnóstico , Masculino , Adulto , Face , Abscesso/diagnóstico , RecidivaRESUMO
We evaluated the primary application of crushed prednisolone combined with hydrocolloid powder for clinically diagnosed peristomal pyoderma gangrenosum (PPG). We present our data on this cohort and follow-up of our previous patients. Of the 23 patients who were commenced on this regime, 18 healed (78%). Twenty-two patients commenced on this regime as the primary treatment for their PPG, and for one, it was a rescue remedy after failed conventional therapy. Four patients with significant medical comorbidities failed to heal and one had their stomal reversal surgery before being fully healed. The proposed treatment regime for PPG is demonstrated to be effective, inexpensive and able to be managed in the patient's usual home environment. In vitro drug release analysis was undertaken, and data are presented to provide further insights into the efficacy of this regime.
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Prednisolona , Pioderma Gangrenoso , Humanos , Prednisolona/uso terapêutico , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/etiologia , Pioderma Gangrenoso/diagnóstico , Pós/uso terapêutico , Liberação Controlada de Fármacos , Resultado do TratamentoRESUMO
INTRODUCTION: Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic skin disease. The diagnosis of PG is mainly based on clinical manifestations. Therefore, the clinical features of PG are important for confirming the diagnosis of this disease. Herein, the clinical data of 2 young males with PG complicated with hematological malignancies were reported, and the literature were reviewed. CASE PRESENTATION: The first case was a 22-year-old male who was admitted due to a systemic rash, headache, and fever. Physical examination showed black scabs on the skins of the extremities, trunk, scalp, and face. Biopsy of the skin lesion showed epidermal edema, spongy formation, neutrophil infiltration, acute and chronic inflammatory cell infiltration in the dermis, showing purulent inflammation with epidermal erosion. The bone marrow biopsy showed obviously active proliferation of nucleated cells, granulocytes at various stages, abnormal morphological neutrophils, and occasionally observed young red blood cells. The diagnosis of PG and chronic myelomonocytic leukemia (CMML-0) was made. The second case was a 28-year-old male who presented a swollen, painful right calf following injury and then developed ulcers on skin and soft tissues. Bone marrow biopsy showed obviously active nucleated cell proliferation, suggesting a myeloid tumor. He was also diagnosed with PG and hematological malignancies. They both received hormone and antiinfection therapy. After treatment, their body temperature, infection, and skin lesions were improved. However, both of them were readmitted and had a poor prognosis. CONCLUSIONS: PG may be associated with hematological malignancies. For patients with typical skin lesions and obvious abnormal blood routines, it is necessary to investigate the possibility of PG with hematological malignancies.
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Neoplasias Hematológicas , Pioderma Gangrenoso , Dermatopatias , Masculino , Humanos , Adulto Jovem , Adulto , Pioderma Gangrenoso/complicações , Pioderma Gangrenoso/diagnóstico , Pele/patologia , Dermatopatias/complicações , Biópsia/efeitos adversos , Neoplasias Hematológicas/complicaçõesRESUMO
This single-center prospective case-control study assessed the association between deep vein thrombosis and healing outcomes in patients with pyoderma gangrenosum.
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Doenças Inflamatórias Intestinais , Pioderma Gangrenoso , Trombose Venosa , Humanos , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , CicatrizaçãoRESUMO
Postoperative pyoderma gangrenosum and peristomal pyoderma gangrenosum are 2 subtypes of pyoderma gangrenosum. The diagnosis is made as a clinicopathologic correlation when assessing a rapidly progressing ulcer with irregular and undermined borders following a surgical procedure, trauma, or the creation of a stoma. Familiarity with the associated risk factors and distinguishing features of these disorders can facilitate prompt recognition, proper diagnosis, and the initiation of treatment. Management usually involves the use of corticosteroids and steroid-sparing agents as immunomodulators to shift the inflammatory neutrophilic dermatoses to chronic noninflammatory wounds and eventual healing.
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Pioderma Gangrenoso , Humanos , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/etiologia , Corticosteroides/uso terapêutico , Cicatrização , Fatores de RiscoRESUMO
Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis with variable clinical features. The classic presentation is an ulceration with an erythematous to violaceous undermined border. Extracutaneous manifestations may occur. Associated systemic diseases include inflammatory bowel disease, inflammatory arthritides, and hematologic disorders. The pathophysiologic mechanism of disease is not completely known but likely related to the cumulative impact of inflammation, immune-mediated neutrophilic dysfunction, and genetic predisposition. Incidence is between 3 and 10 people per million but may be greater due to under recognition. In this article, we will discuss the diagnostic criteria, disease subtypes, systemic associations, and workup.
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Doenças Inflamatórias Intestinais , Pioderma Gangrenoso , Humanos , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/complicações , Inflamação/complicações , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/diagnóstico , Diagnóstico DiferencialRESUMO
Acute febrile neutrophilic dermatosis, or Sweet syndrome, has been described in 1964 and is now considered as a prototypical condition of the group of the neutrophilic dermatoses. Since this time, many clinical conditions have been included in this group and a clinical-pathological classification in 3 subgroups has been proposed. Neutrophilic infiltrates can localize in all internal organs. This defines the neutrophilic disease, which induces difficult diagnostic and therapeutic problems. Autoinflammation is the main pathophysiological mechanism of the neutrophilic dermatoses. There is a special link between myeloid malignancies (leukemia and myelodysplasia) and the neutrophilic dermatoses.
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Dermatite , Pioderma Gangrenoso , Síndrome de Sweet , Humanos , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/patologia , Pele/patologia , Inflamação , Neutrófilos/metabolismo , Neutrófilos/patologiaRESUMO
Hidradenitis suppurativa (HS) is an autoinflammatory skin disorder of the terminal hair follicle, which can present in sporadic, familial, or syndromic form. A classification has been proposed for the latter, distinguishing cases associated with a known genetic condition, with follicular keratinization disorders or with autoinflammatory diseases. This review focuses on the clinical and genetic features of those entities (ie, pyoderma gangrenosum [PG], acne and HS; PG, acne, pyogenic arthritis and HS; psoriatic arthritis, PG, acne and HS; synovitis, acne, pustulosis, hyperostosis, osteitis; and so forth) for which the collective term HS-related autoinflammatory syndromes is proposed.
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Acne Vulgar , Artrite , Hidradenite Supurativa , Hiperostose , Osteíte , Pioderma Gangrenoso , Sinovite , Humanos , Hidradenite Supurativa/diagnóstico , Hidradenite Supurativa/genética , Hidradenite Supurativa/terapia , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/genética , Pioderma Gangrenoso/terapia , Osteíte/complicações , Acne Vulgar/diagnóstico , Acne Vulgar/genética , Acne Vulgar/complicações , Síndrome , Sinovite/complicações , Hiperostose/complicações , Artrite/complicaçõesRESUMO
The term neutrophilic dermatosis encompasses a heterogeneous group of diseases, often associated with an underlying internal noninfectious disease, with an overlapping histopathologic background characterized by perivascular and diffuse neutrophilic infiltrates in one or more layers of the skin; extracutaneous neutrophilic infiltrates may be associated. Neutrophilic dermatoses are not frequent in children and, when they appear in this age group, represent a diagnostic and therapeutic challenge. Apart from the classic neutrophilic dermatoses such as pyoderma gangrenosum, Sweet syndrome, and Behçet disease, a neutrophilic dermatosis can be the presentation of rare genetic diseases of the innate immune system, such as autoinflammatory diseases.
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Dermatite , Pioderma Gangrenoso , Síndrome de Sweet , Humanos , Criança , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/patologia , Pele/patologia , Síndrome de Sweet/diagnóstico , Neutrófilos/patologiaRESUMO
This case illustrates the complex interactions of the immune responses after vaccination and highlights their potential connections to various autoimmune conditions. A 22-year-old man with quiescent ulcerative colitis (UC) presented with abdominal pain, rectal bleeding, and thrombocytopenia 7 days after receiving the third coronavirus disease 2019 mRNA vaccination. Laboratory data confirmed the diagnosis of immune thrombocytopenia. High-dose intravenous immunoglobulin administration boosted the patient's platelet count. Simultaneously, colonoscopy revealed that his UC had relapsed. Although salazosulfapyridine briefly improved his symptoms, his stool frequency worsened one week later. The patient also developed pyoderma gangrenosum. Subsequent treatment with infliximab notably improved both pyoderma gangrenosum and UC.
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Vacinas contra COVID-19 , COVID-19 , Colite Ulcerativa , Púrpura Trombocitopênica Idiopática , Pioderma Gangrenoso , Trombocitopenia , Adulto , Humanos , Masculino , Adulto Jovem , Doença Crônica , Colite Ulcerativa/complicações , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/diagnóstico , COVID-19/complicações , Vacinas contra COVID-19/efeitos adversos , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/etiologia , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/etiologia , Pioderma Gangrenoso/diagnóstico , RecidivaRESUMO
INTRODUCTION: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with an increased risk of mortality compared to the general population. The causes of this increased risk are not well understood. Misdiagnosis is common in PG, and many studies are limited by the inclusion of misdiagnosed cases. The goal of this study was to review autopsy findings, identify causes of death, and identify factors that may worsen outcomes among deceased patients confirmed to have PG. METHODS: Data was retrospectively reviewed from the electronic medical records at five academic hospitals. A search was conducted for deceased patients with a diagnosis of PG who had an autopsy performed between 2010 and 2020. We report a descriptive analysis of 11 patients and their clinical characteristics, causes of death, and autopsy findings. RESULTS: The average age of death was 62.9 years. Seven patients had at least one underlying condition known to be associated with PG including inflammatory bowel disease, inflammatory arthritis, or a hematologic disorder. The most common cause of death was infection (n = 6, 54.5%), followed by pulmonary embolism (n = 3, 27.3%), and myelodysplastic syndrome (n = 2, 18.2%). Six patients (54.5%) were taking systemic steroids at the time of death. CONCLUSION: The development of PG may shorten life expectancy among those with underlying conditions associated with PG, and common treatments for PG may contribute to the risk of fatal complications. Awareness of the risk of infection, thrombosis, and malignancy among those with PG is necessary for proper management. Further research is needed to explore the relationship between PG and thromboembolism.
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Doenças Inflamatórias Intestinais , Pioderma Gangrenoso , Úlcera Cutânea , Humanos , Pessoa de Meia-Idade , Pioderma Gangrenoso/complicações , Pioderma Gangrenoso/diagnóstico , Estudos Retrospectivos , AutopsiaRESUMO
Pyoderma gangrenosum is a rare disease in childhood. Extra-cutaneous manifestations are uncommon in pyoderma gangrenosum, even more so in children, with only a few cases reported in the literature. We present the case of a pediatric patient with pyoderma gangrenosum and associated pulmonary involvement. In this case, the diagnosis was delayed leading to late initiation of therapy, emphasizing the importance of maintaining a high level of suspicion for this diagnosis.
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Pioderma Gangrenoso , Humanos , Criança , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , Pioderma Gangrenoso/complicações , Biópsia/efeitos adversos , Tomografia Computadorizada por Raios XRESUMO
Pyoderma gangrenosum (PG) is a rare autoinflammatory disorder falling under the spectrum of neutrophilic dermatosis, characterized by distinctive skin ulceration which is non-infective, non-neoplastic and usually with no primary vasculitis. PG lesions are notorious for relapse and hence require multiple trials of medications often with prolonged and concomitant use of steroids. Due to lack of evidence-based studies on effective treatment options for PG, we have presented three isolated biopsy-proven PG cases who were successfully treated with Tofacitinib, a Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway inhibitor, without relapse in follow up.