Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that causes pustules and ulcerations (1). Its diagnosis and appropriate management are often delayed because of its rarity and the presence of numerous clinical variants. Although corticosteroids remain the primary first-line treatment for severe forms of PG, the use of Janus kinase inhibitors (JAKis) is promising (2, 3). To date, 31 cases of PG treated with JAKi have been reported and are presented in Table 1 (4–24). In this case report, the authors describe a 20-year-old man with long-standing, non-healing, and painful facial ulcers that were not responsive to multiple antibiotics and serial wound debridement. Treatment with Upadacitinib hydrate and a tapering course of oral prednisolone was initiated. However, FPG is a rare dermatological pathology lacking characteristic distinguishing features, making treatment challenging (25).

We report the case of a 20-year-old man who presented with a 4-month history of painful facial skin ulcers on 18 March 2025 (Figures 1A,B). The lesion appeared after excision of facial cysts. The patient had a history of eczema and acne, with no family history of similar conditions and psychosocial issues. On 10 March 2025, a biopsy suggested an infectious granuloma; however, negative acid-fast and periodic acid–Schiff stains were negative (Figure 1I). During previous evaluations of the ulcers, next-generation sequencing suggested the presence of Streptococcus pneumoniae and Klebsiella pneumoniae; however, antimicrobial therapy was ineffective. A second biopsy was conducted on 27 March 2025. Histopathological analysis of a skin-biopsy specimen obtained from the lesion border revealed a diffuse mixed inflammatory cell infiltrate. Cultures and polymerase chain reaction tests for deep mycosis and mycobacterial infections were negative. No acid-fast bacilli, fungi, or bacteria were identified using acid-fast or periodic acid—Schiff stains. Test results for herpes simplex virus 1, herpes simplex virus 2, interferon-gamma release assays, syphilis, human immunodeficiency virus, and hepatitis B DNA all showed no abnormalities. Additionally, tests for rheumatologic conditions and immunodeficiency were negative. At the current presentation, physical examination revealed a skin ulcer with a violaceous border on the right lower portion of the face (Figures 1C–E). We made the diagnosis of facial pyoderma gangrenosum (FPG). The patient was treated with oral prednisolone (0.5 mg/kg, six tablets) monotherapy once daily for 2 weeks. However, as the ulcer enlarged, treatment was escalated to combination therapy with Upadacitinib (15 mg once daily) for 8 weeks. Treatment with Upadacitinib hydrate and a tapering course of oral prednisolone was initiated. The patient took six tablets of prednisone orally daily for 2 weeks, took four tablets for 2 weeks, and reduced the dosage by one tablet per week until the medication was discontinued. By 3 April 2025, the majority of the ulcers on the left mandible and the left neck had healed. The skin lesion started to subside 1 month after the start of treatment. Our case responded well to Upadacitinib without adverse events. After 4 months, the patient reported complete resolution of the lesions (Figures 1F–H). At present, the patient is still under our follow-up schedule.

PG is a debilitating skin disease marked by idiopathic neutrophil infiltration that causes the destruction of tissue and ulceration (24). Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG involves dysregulation of both innate and adaptive immunity (1), leading to a neutrophil-rich autoinflammatory process with the elevation of multiple cytokines (15, 26). Some of these cytokines act through the JAK/STAT pathway (3). The importance of the JAK/STAT pathway in PG has also been demonstrated through immunohistochemistry in skin biopsy specimens (27). The predisposition of PG is not well understood. Drug induction and the postoperative period are two potential triggers. In our case, the cause of FPG is due to the excision of left-sided facial cysts.

FPG is a rare subtype of PG (25). PG needs to be differentiated from infections, such as mycobacterial cellulitis, syphilitic granulomatous ulcers, and scrofuloderma; lupus vulgaris; malignancies; and vasculitis (2). It is often associated with various other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis (28). It may be associated with systemic inflammatory conditions, including inflammatory bowel disease (IBD), rheumatoid arthritis, or vasculitis, as well as leukemia or hepatitis. It may also be present in the setting of autoinflammatory syndromes, such as pyogenic arthritis, PG, and acne; PG, acne, and suppurative hidradenitis; and pyogenic arthritis, PG, acne, and suppurative hidradenitis, and in a small proportion of synovitis, acne, pustulosis, hyperostosis, and osteitis cases (28).

Diagnosing PG is challenging since there are no pathognomonic laboratory parameters or histopathological features (13). In 2018, a new Delphi consensus was published on the diagnostic criteria for PG, stating that diagnosis could be made by using one major and several minor criteria. The major criterion was neutrophilic infiltration at the ulcer edge on biopsy. The eight minor criteria are 1) the exclusion of infection; 2) a positive pathergy test; 3) a history of IBD or inflammatory arthritis; 4) the evolution of pustules, papules, or vesicles into ulcers within four days; 5) erythema, undermined borders, and tenderness around ulcers; 6) multiple ulcers with at least one location on the extensor surface of the lower leg; 7) cribriform or “wrinkled paper” scars at the site of healed ulcers; and 8) a reduction in ulcer size within one month after treatment with immunosuppressive drugs (29).

Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) followed by the addition of more slow-acting immunosuppressive drugs with superior adverse event profiles, including biologics, intravenous immunoglobulin (30), and JAK inhibitors (3, 28, 31). Our case and analysis of the previously published cases demonstrate JAKi as an effective treatment option for PG (3) (Table 1). Tofacitinib (a non-selective JAK inhibitor), ruxolitinib (JAK-1/2 inhibitor), and Upadacitinib (JAK-1 inhibitor) have been reported to be successful in treating PG in a handful of reported cases (10). Patients responded in a relatively brief period of time with few reported adverse events (22).

Patients documented to be treated with JAKis (Table 1) had a mean age of 55.2 years (range: 20–89 years) and consisted of 25.8% males and 74.2% females (Table 1). PG presented as ulcerations on the lower extremities in approximately 74% (23/31) of cases. Treatments were categorized as JAKi treatment combined with concomitant medications (11/31, 35.5%) or JAKi monotherapy without concomitant medications (20/31, 64.5%). Among JAKis, the most commonly used was tofacitinib (8/20, 40.0%), followed by baricitinib (5/20, 25%) and upadacitinib (5/20, 25%). Among JAKis used with concomitant medication, tofacitinib (8/11, 72.7%) was the most common, followed by upadacitinib (2/11, 18.2%) and ruxolitinib (1/11, 9.1%). The most common concomitant medication used was systemic corticosteroids (6/11, 54.5%).

While the management of PG is challenging because of the lack of standardized evidence-based treatments, notable advancements are being made in its identification and management. In our case, PG initially stabilized and subsequently decreased in severity under treatment with a JAKi and a tapering course of oral prednisolone.