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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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.

UNASSIGNED: Pyoderma gangrenosum (PG) following a primary total knee arthroplasty (TKA) surgery is extremely rare, with very few cases reported in the literature.
UNASSIGNED: We report our clinical experience of a 65-year-old female who developed PG following a primary TKA surgery. Corticosteroids and local wound care with vacuum-assisted closure dressing helped achieve rapid improvement in the wound condition.
UNASSIGNED: Post-surgical PG in TKA can be challenging with limited evidence for its definitive treatment. A high degree of suspicion and a multidisciplinary management approach will help in the timely diagnosis and optimization of treatment for this condition.

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Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that is associated with systemic inflammatory conditions. Currently, there is no universally accepted standard therapy for PG, but immunosuppressive (IS) treatment seems essential. We report a patient here who was successfully treated with tofacitinib despite being PG-refractory to multiple anti-tumor necrosis factor alpha (anti-TNF) therapies and conventional IS. In addition, we performed a comprehensive review of all cases of PG treated with JAK inhibitors. We identified 27 cases treated with JAK inhibitors. Approximately 80% of the patients achieved complete recovery within a median of 12 weeks, even though 17 patients (63%) had received biologics before JAKinib treatment. Notably, this recovery could appear as early as 2 weeks. JAK inhibitors may prove useful in the future, particularly for treating immunosuppressive and steroid-resistant pyoderma gangrenosum, according to recent case reports.

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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.