PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome is a rare autoinflammatory disorder often arising in pediatric patients. We present a case of an 18-year-old female with a past medical history of growth failure, immunoglobulin A nephropathy, and inflammatory arthritis who presented to a pediatric dermatology clinic with findings of acne, psoriasiform dermatitis, and hidradenitis suppurativa, whose clinical, genetic, and laboratory findings were most consistent with PAMI syndrome. We conducted a literature review to better characterize this rare condition in the context of dermatologic findings. Recognition of the distinctive skin findings seen in PAMI syndrome can help distinguish it from other inflammatory disorders, enabling expedited diagnosis and treatment.

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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.

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 (PG) is a rare disease characterized by ulcerative cutaneous lesions that can occur postoperatively and is often associated with autoimmune disorders. PG is diagnosed by excluding other conditions that can cause ulcerations, such as infections, which may also result in immunosuppressive treatment delays and suboptimal wound care. Operative debridement of wounds has traditionally been avoided in the acute setting secondary to pathergy. This article presents a case of extensive breast PG that was successfully treated with surgical debridement, porcine-derived extracellular matrix, and negative pressure wound therapy while on systemic immunosuppressive therapy.

UNASSIGNED: Postsurgical pyoderma gangrenosum (PSPG) is a highly uncommon and unpredictable wound healing complication. Rapid progression of ulcers at incisions can cause unfettered dehiscence. Most commonly, PSPG involves breast procedures; however, in this work, we detail a case of a patient who developed PSPG 10 days postoperatively after penile inversion vaginoplasty.
UNASSIGNED: The patient in this case underwent a penile inversion vaginoplasty with orchiectomy in the standard fashion. She had no risk factors for PSPG. Following an uncomplicated hospital stay, the patient developed difficulty with pain control and increasing serous drainage on the 10th postoperative day. On readmission, the patient was found to have developed large, mildly purulent ulcers throughout the perineal wound edges. On exam under anesthesia, the neovaginal canal was found to be patent and intact. The dehisced portions of the incisions were left open and redressed with occlusive bismuth-petrolatum dressing. Dermatology was promptly consulted with suspicion for PSPG. The patient was started on an 18-day prednisone taper with cyclosporine, along with doxycycline and ciprofloxacin.
UNASSIGNED: After 5 days of immunosuppressive treatment, the ulcers visibly converted to healthy granulation tissue and were no longer actively purulent. Following another washout, the dehisced wound edges were reapproximated. At follow-up, the patient had no evidence of PSPG recurrence and continued dilating on schedule. Our patient recovered from PSPG without further complications and a satisfactory aesthetic result.
UNASSIGNED: This unique case highlights the importance of prompt dermatological consultation, immunosuppression, and avoidance of further pathergy in the setting of suspicion for PSPG.

We report a case of a 54-year-old female diagnosed with HIV and antiretroviral therapy (ART) for the same. Seven years ago, she suffered from fever, cough and weight loss, was diagnosed with pulmonary tuberculosis and also seropositive for HIV. She suffered from Herpes Zoster infection, after which her ART regimen was changed to TLD (tenofovir, lamivudine and dolutegravir). The patient presented with two episodes of pyoderma gangrenosum (PG), which were biopsy-proven, corresponding to a rise in CD4 counts above 500. She responded to glucocorticoids, both systemic and topical.

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.

Pyoderma gangrenosum (PG) is an extremely rare case of sterile necrotic ulcerative disease associated with malnutrition as a predisposition factor. It is unclear, though, whether dilated cardiomyopathy, which affects blood flow and results in stenosis in the arteries, could play a role as an etiology. In this study, a case of pyoderma gangrenosum in a 10-year-old boy complicated by dilated cardiomyopathy, a previous history of cerebrovascular disease, and a malnourished condition were reported. The patient was reported to have exudative necrotic lesions in both legs. Lesions began as small, multiple, itchy lesions on both legs, which later became blisters and scuffed, and progressed into painful, peeled-off lesions with pus, bleeding, redness around lesions, and maggots within a month. A high fever was an accompanying symptom. The multidisciplinary team was involved to provide a comprehensive treatment for this patient. Antibiotics and necrotomy debridement were performed several times. Anticoagulant treatment was indicated as the coagulation markers were increased and echocardiography suggested thrombus in the left ventricle. The underlying condition that increases the risk of pyoderma gangrenosum should be corrected. The patient was discharged after a clinical improvement, although the continuation of outpatient monitoring was required. Our report suggests that a chronic condition of dilated cardiomyopathy that affects normal blood flow leads to malnutrition, the formation of thrombus, and stenosis of a peripheral artery, all of which contributed to pyoderma gangrenosum. Therefore, early surgical treatment, antibiotic administration, and anticoagulant treatment were recommended.

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.