Chronic ulcer in a patient with essential thrombocythemia taking hydroxyurea.

Chronic skin ulcers in patients with suspected pyoderma gangrenosum can, on closer inspection and further workup, have a different cause. Recognition of key features on clinical examination such as the presence of atrophie blanche is key to avoid misdiagnosis of pyoderma gangrenosum and its subsequent treatment with high-dose corticosteroids and other immunosuppressive medications.

[Pyoderma gangrenosum after orthopedic conservative treatment]. / Pyoderma gangrenosum après traitement conservateur orthopédique.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Half of the cases are associated with an immune dysfunction and are frequently triggered by pathergy such as a tissular aggression via surgery or burn wounds. A patient with ulcerative colitis presented a PG at the site of an iontophoresis patch for tendinopathy. Treatment in a specialized burn center, corticosteroid therapy and adapted local care contributed to a favourable evolution. PG remains a diagnosis of exclusion and inflammatory phenomena must be differentiated from infectious causes such as necrotizing fasciitis to initiate immunosuppressive treatment. Being rare and difficult to diagnose and to treat as well as associated with potentially severe sequelae, a multidisciplinary team is required for the management of PG.

Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare. Il est, dans la moitié des cas, associé à une maladie dysimmunitaire et il est fréquemment déclenché par un phénomène de pathergie, défini comme une agression tissulaire par une intervention chirurgicale ou encore une brûlure. Une patiente avec une rectocolite ulcéro-hémorragique a développé un PG sur le site d'application d'un patch d'ionophorèse pour une tendinopathie. Un traitement par une corticothérapie, un traitement immunosuppresseur local et des soins locaux adaptés ont permis une évolution favorable. Le PG reste un diagnostic d'exclusion et les phénomènes inflammatoires doivent être différenciés de phénomènes infectieux, comme la fasciite nécrosante, afin d'initier rapidement des immunosuppresseurs. Comme il s'agit d'une pathologie rare avec un diagnostic difficile, que des séquelles peuvent être catastrophiques et qu'un traitement immunosuppresseur complexe doit être instauré, une équipe pluridisciplinaire est requise pour la prise en charge de cette pathologie.

Other Wounds Encountered in Clinical Practice.

This chapter delves into uncommon wounds including pyoderma gangrenosum, sickle cell disease ulcers, vasculitic wounds, Martorell hypertensive ischemic leg ulcers, and malignant ulcers. Emphasizing a multidisciplinary approach, it covers diagnostics, treatments, and challenges, with case studies illustrating complexities in managing these conditions. The discussion extends to radiation-related wounds, underscoring the need for patient-centered care, interdisciplinary collaboration, and realistic goal setting. Overall, the chapter navigates the intricacies of uncommon wounds, emphasizing the importance of tailored approaches for improved outcomes in patients with diverse underlying conditions.

An Unusual Case Presentation of Non-Pathergy Induced Severe Pyoderma Gangrenosum Localized to the Perianal and Intragluteal Regions.

Pyoderma gangrenosum is a rare neutrophil based disorder presenting as a painful sterile ulcer usually below the knee. The disease is also linked to comorbid conditions including inflammatory bowel disease (IBD) and pathergy. Mainstay treatment begins with high dose steroids, either topical for limited disease or systemic for extensive or rapidly progressing disease. This report presents a patient who had perianal and intragluteal cleft pyoderma gangrenosum, not related to IBD or pathergy, where high dose steroids were contraindicated due to history of steroid induced psychosis.

Adjunctive hyperbaric oxygen treatment for challenging pyoderma gangrenosum cases.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, difficult-to-treat neutrophilic ulcerative cutaneous condition that severely impacts those affected. Treatment options for PG are limited, and disease remission is not guaranteed. Hyperbaric oxygen treatment is a potential therapeutic option for treating various ulcerative conditions not frequently utilized for PG. CASE REPORT: We present a case of a patient with treatment-resistant PG who achieved remission with adjunctive HBOT, and then later had difficulty achieving remission without HBOT during a future flare. DISCUSSION: HBOT should be more readily considered as a treatment option for those with PG.

[Ulcerative colitis diagnosed with facial pyoderma gangrenosum: a case report].

Pyoderma gangrenosum (PG) is a sterile inflammatory skin condition that is frequently associated with immune-related diseases, including inflammatory bowel disease (IBD). PG causes noninfectious ulcers. Facial PG is uncommon while PG usually occurs on the trunk and lower limbs. Herein, we report a case of a male teenager with fever, pustules, ulcers, and necrosis on both cheeks. He was initially diagnosed with complicated acne with bacterial infection, but the condition progressed to subcutaneous ulcers despite treatment. Biopsy revealed inflammatory lesions in dermal and subcutaneous tissue with neutrophil infiltration, consistent with PG. Although lacking typical IBD symptoms, blood tests revealed anemia and positive fecal occult blood. Sigmoidoscopy revealed inflammation, ulcers, and pseudopolyps in the colon and rectum, thereby diagnosing ulcerative colitis (UC). After treating PG and UC with prednisolone and skin grafts, golimumab was prescribed. The patient is now in remission. Necrotic tissue buildup can complicate closure in PG cases;this emphasizes the need for effective IBD treatment to facilitate procedures such as skin grafts.

Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome: a single-institution case series with a focus on management.

BACKGROUND: Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The management of PASH syndrome is difficult, with no consensus on treatment guidelines. Since PASH syndrome can increase morbidity and adversely impact quality of life, better characterization of effective therapies is needed. METHODS: A retrospective cohort study was conducted to identify all patients with pyoderma gangrenosum (PG) treated at The Ohio State University Wexner Medical Center between 2015 and 2021. PG diagnosis was confirmed via PARACELSUS score. Subsequent chart review identified eight patients with concomitant hidradenitis suppurativa (HS) and acne who were clinically diagnosed with PASH syndrome. RESULTS: Eight patients were clinically diagnosed with PASH syndrome based on their clinical presentation at our institution. Seven patients had failed some type of medical therapy prior to presentation, including topical corticosteroids, oral corticosteroids, oral antibiotics, and biologics. One patient had also tried surgical drainage at an outside institution. Six patients were effectively treated with biologics, usually in combination with other therapies. One patient experienced improvement of her skin lesions after diagnosis and treatment of her underlying hematologic malignancy. CONCLUSIONS: Medical management with biologics in combination with corticosteroids and/or antibiotics was effective in the management of most patients. Diagnosis and treatment of an underlying condition should be prioritized in refractory cases. If workup is negative, surgical management may be considered. Further investigation with a greater number of patients is required to develop management guidelines for PASH syndrome.

The role of split-thickness skin grafting in the treatment of vasculitic and pyoderma gangrenosum ulcers in a multidisciplinary wound centre.

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.

A confounding clinically aggressive case of necrotizing granulomatous and suppurative dermatitis.

Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on the trunk. Herein, we report a clinically confounding case of disseminated superficial granulomatous pyoderma gangrenosum in a patient with a 5-year history of painful and chronic ulcerations of the bilateral upper extremities and face in a sun exposed distribution. This was a diagnostically challenging case due to the treatment-refractory nature of our patient's skin lesions and the atypical clinical and histologic presentations encountered. We review our clinical decision process and acknowledge other entities that were considered during the clinical course of this case. Additionally, we discuss the lack of responsiveness to various treatment options with eventual successful clearance of this patient's active skin disease with initiation of adalimumab.

Janus kinase inhibitors in the treatment of pyoderma gangrenosum: case report and review.

Pyoderma gangrenosum (PG) is a rare inflammatory dermatologic condition with neutrophilic infiltration of the skin that causes pustules and ulcerations. Janus kinase (JAK) inhibitors are immunomodulating agents that have been recently described in the literature as an effective treatment for PG. We describe a patient with PG on the lower extremities successfully treated with baricitinib. We also conducted a narrative review of the literature of PG patients treated with JAK inhibitors who were refractory to other treatments.

Monoclonal gammopathy in the setting of Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.

Pyoderma Gangrenosum of the Genitalia, Anus, and Perineum: Two Case Reports and a Review of Published Cases.

ABSTRACT: Pyoderma gangrenosum is an inflammatory skin disease that presents with rapidly progressive ulcers with violaceous, undermined borders. Despite most commonly affecting the lower extremities, pyoderma gangrenosum can rarely present in the genital, anal, and perineal regions. We describe 2 cases and report a review of published cases.