Penile Pyoderma Gangrenosum, a Rare Encounter in STI Healthcare: A Case Report and a Review of the Literature.
Ontology highlight
ABSTRACT:
SUBMITTER: Salado-Rasmussen K
PROVIDER: S-EPMC11000657 | biostudies-literature | 2024 Mar
REPOSITORIES: biostudies-literature
Publications
Penile Pyoderma Gangrenosum, a Rare Encounter in STI Healthcare: A Case Report and a Review of the Literature.
Salado-Rasmussen Kirsten K Alsing Kasper Køhler KK Mårtensson Nina Løth NL Burian Ewa Anna EA Larsen Helle Kiellberg HK
Acta dermato-venereologica 20240329
Similar Datasets
Project description:Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome is a rare inflammatory disease characterized by pyoderma gangrenosum (PG), mild to severe facial acne, and hidradenitis suppurativa (HS). It only affects the skin and represents cutaneous characteristics of a spectrum of autoinflammation. Lack of pyogenic sterile arthritis (PA) distinguishes the pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome from pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PA-PASH), pyoderma gangrenosum, acne, hidradenitis suppurtiva, and ankylosing spondylitis (PASS), and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndromes. The exact etiology and pathogenesis of PASH syndrome remain unknown. Both PG and HS are contained in the spectrum of neutrophilic dermatitis, which is considered as an autoinflammatory syndrome. From a pathophysiological point of view, they show similar mechanisms, including neutrophil-rich cutaneous infiltration and overexpression of the interleukin-1 (IL-1) family. These findings provide guidance for these intractable diseases. In this review, we described a case of PASH syndrome in a patient who initially failed to respond to immunosuppressive treatment but responded to a combination of colchicine and thalidomide. We reviewed the relevant literature that focuses on PASH syndrome management.
| S-EPMC8987973 | biostudies-literature
Project description:BackgroundPocket complications are common after cardiac implantable electronic device implantation. We report a rare case of pyoderma gangrenosum (PG) complicating a permanent pacemaker implantation, and the first literature review of 10 published cases.Case summaryFive days after pacemaker implantation for heart failure and 2:1 atrioventricular block, a 93-year-old man had pain in the scar and bleeding on contact. Two days later, he had fever, inflammatory syndrome, and a necrotic 7-cm wound. The pacemaker was removed and he was started on antibiotics. Due to a lack of bacterial growth in samples, PG (a rare aseptic, destructive inflammatory cutaneous condition) was suspected, and histology was compatible with this diagnosis. High-dose corticosteroids vastly improved his condition within 1 week, and after 2 months of decreasing-dose corticosteroid therapy, complete healing and normalization of the inflammatory syndrome were observed.DiscussionPyoderma gangrenosum should be considered if there is aseptic skin ulceration that is not controlled by antibiotic treatment. The first-line treatment for PG is high-dose systemic corticosteroids.
| S-EPMC7180522 | biostudies-literature
Project description:Pyoderma gangrenosum is a challenging disease to manage, due in part to the lack of approved treatment therapies. Recently, the emergence of biologic agents has expanded treatment options, with tumour necrosis factor alpha inhibitors being the best supported in the literature. In our report, we present a 50-year-old female with pyoderma gangrenosum who was successfully treated with the anti-interleukin-17 biologic agent, secukinumab, after failing other systemic therapies.
| S-EPMC7370553 | biostudies-literature
Project description:Pyoderma gangrenosum is a rare skin necrotizing disease that can arise on a site of surgical trauma. Its pathogenesis has recently been related to dysregulation of the immune system, with inflammatory bowel disease representing the most commonly underlying systemic conditions. Several authors have also reported an association with solid malignancies (especially gastrointestinal and breast cancer). We describe the case of a 39-year-old patient diagnosed with a locally advanced, triple-negative breast cancer who developed a pyoderma gangrenosum on the surgical wound after a CVC implant with systemic complications. As the diagnosis and management of postsurgical pyoderma gangrenosum can be challenging for clinicians, underlying conditions as autoimmune disease and solid tumors have to be considered in order to guide treatment.
| S-EPMC7983549 | biostudies-literature
Project description:ObjectivesThis study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.MethodsThe clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.ResultsA total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The PSTPIP1 A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 vs. 83%), whereas arthritis was less common (50 vs. 98.1%). Steroid and/or biological agents were effective in most patients.ConclusionsThe rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.
| S-EPMC8569796 | biostudies-literature
Project description:IntroductionSkin ulcers can be challenging to diagnose and manage, particularly with comorbid autoimmune and gastrointestinal diseases. Occam's razor encourages the simplest explanation to guide care, but reconsideration must occur when intervention proves futile.Case presentationWe report the case of a 70-year-old male, with a 17-year history of expanding pretibial skin ulcer, presumed by prior care providers to be pyoderma gangrenosum related to Crohn's disease. A surgical biopsy performed upon presentation to our institution revealed basal cell carcinoma of the skin, invasive to the proximal tibia with associated deep infection, prompting transfemoral amputation.ConclusionThis report is written as a reminder to reconsider a diagnosis and consider seeking additional expertise when a patient's condition progressively worsens despite intervention. Earlier diagnosis likely would have facilitated therapeutic limb salvage care.
| S-EPMC10870126 | biostudies-literature
Project description:BackgroundPyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare autosomal dominant genetic disease characterized by severe autoimmune inflammation, caused by mutations in the PSTPIP1 gene. Due to PAPA heterogeneous clinical manifestation, misdiagnosis or delayed diagnoses are difficult to avoid. With the use of whole-exome sequencing, we identified a missense mutation in the PSTPIP1 gene in a Chinese family. To the best of our knowledge, this is the first case of PAPA reported in China.Case summaryA 9-year-old boy suffered from recurrent aseptic pyogenic arthritis triggered by minor trauma or few obvious predisposing causes for more than 3 years. Pyogenic arthritis occurred every 3-5 mo, affecting his knees, elbows, and ankle joints. Treatments, such as glucocorticoids, antibiotics, even surgeries could alleviate joints pain and swelling to some extent but could not inhibit the recurrence of arthritis. Similar symptoms were present in his younger brother but not in his parents. According to the whole-exome sequencing, a missense mutation in exon 11 of the PSTPIP1 gene (c.748G>C; p.E250Q) was detected in the boy, his younger brother and his father. Taking into account the similar phenotypic features with PAPA syndrome reported previously, we confirmed a diagnosis of PAPA syndrome for the family.ConclusionIn this case, a missense mutation (c.748G>C; p.E250Q) in PSTPIP1 gene was identified in a Chinese family with PAPA syndrome. Previous studies emphasize the fact that PAPA syndrome is hard to diagnose just through the clinical manifestations owing to its heterogeneous expression. Genetic testing is an effectual auxiliary diagnostic method, especially in the early stages of pyogenic arthritis. Only if we have a deep understanding and rich experience of this rare disease can we make a prompt diagnosis, develop the best clinical treatment plan, and give good fertility guidance.
| S-EPMC8362586 | biostudies-literature
Project description:Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition of an unknown etiology and is often associated with immune diseases. However, PG rarely shows visceral involvement, especially in the kidney. A 20-year-old female presented with pedal edema and skin ulceration of both lower limbs. The skin lesion began as an erythematous plaque and then became a blister. She also complained of abdominal distension and a decreasing urine volume. Laboratory data showed high proteinuria, hypoalbuminemia and hyperlipidemia. Her skin and kidney were biopsied. The pathological results indicated PG and immunoglobulin A (IgA) nephropathy. The patient was finally cured with prednisolone in combination with cyclosporine A (CsA).
| S-EPMC6232568 | biostudies-other
Project description:IntroductionPyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of non-infectious etiology. Cocaine-induced PG (CIPG) is a documented clinical variant.Case presentationWe report an exceptional case of cocaine-induced PG flare unresponsive to conventional treatment in the context of positive COVID status. A 41year-old male with past medical history of recent COVID infection, pyoderma gangrenosum and chronic cocaine abuse presented with acutely worsening multifocal ulcerations covering multiple limbs approximately 30% body surface area (BSA) one day after cocaine use. After hospitalization for ten days with no improvement in cutaneous symptoms, he was transferred to a burn center for disease control with biologics.DiscussionThe previous temporal relationship between disease outbreak and cocaine consumption and improvement after its discontinuation no longer remained in the setting of COVID positive status. This is the first case in literature of extensive and treatment-refractory PG in a COVID-positive patient with recent cocaine use.ConclusionThis case highlights the importance of further investigation on the connection between COVID infection and PG and the need for establishing treatment guidelines for PG.
| S-EPMC9113765 | biostudies-literature
Project description:Pyoderma gangrenosum (PG) is a rare, destructive inflammatory skin disease of which a painful nodule or pustule breaks down to form a progressively enlarging ulcer. Ulcerations associated with PG may occur after trauma or injury to the skin. The etiology has not been clearly elucidated. Our report described a PG patient with a heterozygous splice-donor-site mutation in NFKB1 (c.730+5G>A) causing the absence of exon 8 and the formation of truncated p105 (p.Asp191_Lys244delinsGlu; p105delEx8), which led to distinct symptoms of high fever and excessive inflammation in wound area after routine surgical procedures. The functional analysis showed that the variant caused reduced phosphorylation of p105 and resulted in the decreased processing of p105 to p50. We conclude that the patient's symptoms were caused by dysregulation of the NF-κB signaling pathway.
| S-EPMC8383449 | biostudies-literature