Pyoderma Gangrenosum in a Patient With Dubowitz Syndrome: a New Comorbidity?

Bevilacqua, Matteo; Granieri, Giammarco; Fidanzi, Cristian; Salvia, Giorgia; Margiotta, Flavia Manzo; Michelucci, Alessandra; Romanelli, Marco; Dini, Valentina

doi:10.25270/wnds/22051

Cited by 2 publications

(5 citation statements)

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“…It is essential to consider other potential causes of ulcers, including infectious etiologies, vasculitic conditions, and malignancies. Close collaboration between dermatologists, geneticists, and other specialists is crucial to reaching an accurate diagnosis and providing appropriate management for patients with Dubowitz syndrome and suspected or confirmed pyoderma gangrenosum [11].…”

Section: Differential Diagnosis Of Pyoderma Gangrenosum In Dubowitz S...mentioning

confidence: 99%

“…Lesions often have undermined borders and may show a predilection for certain anatomical sites, such as the lower extremities. However, the clinical presentation can be variable, and the association between Dubowitz syndrome and pyoderma gangrenosum needs further exploration to establish a more comprehensive understanding of its specific clinical features (Table 1) [11].…”

Section: Clinical Characteristics Of Pyoderma Gangrenosum In Dubowitz...mentioning

confidence: 99%

“…Examples may include systemic immunosuppressive agents such as corticosteroids, azathioprine, or mycophenolate mofetil. Additionally, case studies may explore biological therapies, such as anti-TNF-alpha agents, which have shown promise in managing pyoderma gangrenosum [11,26].…”

Section: Case Studies or Examples Of Treatment Approachesmentioning

confidence: 99%

“…Understanding the cutaneous manifestations of Dubowitz syndrome, including Pyoderma gangrenosum, is crucial for the appropriate diagnosis, management, and comprehensive care of individuals with this complex genetic disorder. In the following sections, we will explore Pyoderma gangrenosum in more detail, including its epidemiology, pathogenesis, diagnosis, and treatment considerations within the context of Dubowitz syndrome [11].…”

Section: Introduction To Cutaneous Manifestations In Dubowitz Syndromementioning

confidence: 99%

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Pyoderma Gangrenosum: A Challenging Cutaneous Manifestation in Dubowitz Syndrome

Ghode¹,

Kenjale²,

Heda³

et al. 2023

Cureus

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Pyoderma gangrenosum (PG) is a challenging cutaneous manifestation associated with Dubowitz syndrome, a rare genetic disorder characterized by multiple congenital anomalies, developmental delay, and distinctive facial features. This review article aims to provide a comprehensive overview of the association between Dubowitz syndrome and pyoderma gangrenosum, emphasizing the clinical presentation, challenges in diagnosis and management, and potential underlying mechanisms. A comprehensive literature search was conducted to gather relevant studies, and inclusion and exclusion criteria were applied to select appropriate articles. The association between Dubowitz syndrome and pyoderma gangrenosum has been documented in reported cases and studies. Clinical characteristics of Pyoderma gangrenosum in Dubowitz syndrome include painful necrotic ulcers with undermined borders. Diagnosing pyoderma gangrenosum in the context of Dubowitz syndrome can be challenging due to the overlapping clinical features and complexities associated with the syndrome. Managing pyoderma gangrenosum involves a multidisciplinary approach, with general principles of wound care, systemic therapy, and pain management. Specific considerations for treating pyoderma gangrenosum in Dubowitz syndrome include collaboration among specialists and careful monitoring. Future directions for management include further research to understand the underlying mechanisms and develop targeted therapies. Recognizing and addressing pyoderma gangrenosum in Dubowitz syndrome is crucial for optimal patient care. This review enhances awareness among healthcare professionals and provides insights for improving diagnosis, management, and treatment outcomes for individuals with this challenging combination of conditions.

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Section: Differential Diagnosis Of Pyoderma Gangrenosum In Dubowitz S...mentioning

confidence: 99%

Section: Clinical Characteristics Of Pyoderma Gangrenosum In Dubowitz...mentioning

confidence: 99%

Section: Case Studies or Examples Of Treatment Approachesmentioning

confidence: 99%

Section: Introduction To Cutaneous Manifestations In Dubowitz Syndromementioning

confidence: 99%

See 2 more Smart Citations

Pyoderma Gangrenosum: A Challenging Cutaneous Manifestation in Dubowitz Syndrome

Ghode¹,

Kenjale²,

Heda³

et al. 2023

Cureus

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“…The ulcerative phenotype is the most prevalent form, primarily affecting the lower limbs. PG is frequently associated with IBD, rheumatoid arthritis (RA), seronegative arthritis, monoclonal gammopathy, and hematological malignancies [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

The Role of Ultra-High-Frequency Ultrasound in Pyoderma Gangrenosum: New Insights in Pathophysiology and Diagnosis

Granieri,

Michelucci,

Manzo Margiotta

et al. 2023

Diagnostics

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Pyoderma gangrenosum (PG) is a neutrophilic dermatological disease, whose pathogenesis is still poorly clarified. Because of the lack of validated criteria for diagnosis and response, PG treatment is still challenging and should be differentiated in the inflammatory and non-inflammatory phases. Our study aimed to provide a new semi-quantitative approach for PG diagnosis and monitoring, identifying ultra-high-frequency ultrasound (UHFUS) early biomarkers associated with the transition between the two phases. We enrolled 13 patients affected by painful PG lesions evaluated during the inflammatory phase (T0) and during the non-inflammatory phase (T1): pain was measured by the Visual Analogue Scale (VAS); clinical features were recorded through digital photography; epidermis and dermis ultrasound (US) characteristics were evaluated by UHFUS examination with a 70 MHz probe (Vevo MD® FUJIFILM VisualSonics). In T1 UHFUS examination, the presence of hyperechoic oval structures was lower compared to T0 (p value < 0.05). An hyperechogenic structure within the oval structure, suggestive of a hair tract, was evident in T0 and absent in T1 (p value < 0.05). In T0, blood vessels appear as U-shaped and V-shaped anechoic structures with a predominance of U-shaped vessels (p value < 0.05) compared to the more regular distribution found in T1. Finding early biomarkers of the transition from the inflammatory to the non-inflammatory phase could provide new insight in terms of therapeutic decision making and response monitoring. The differences found by this study suggest a potential use of UHFUS for the development of an objective standardized staging method. Further investigations will be necessary to confirm our preliminary results, thus providing a turning point in PG early detection, differential diagnosis and treatment monitoring.

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Pyoderma Gangrenosum in a Patient With Dubowitz Syndrome: a New Comorbidity?

Cited by 2 publications

References 0 publications

Pyoderma Gangrenosum: A Challenging Cutaneous Manifestation in Dubowitz Syndrome

Pyoderma Gangrenosum: A Challenging Cutaneous Manifestation in Dubowitz Syndrome

The Role of Ultra-High-Frequency Ultrasound in Pyoderma Gangrenosum: New Insights in Pathophysiology and Diagnosis

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