Hrev_master [page 74] [Emergency Care Journal 2024; 20:12790] Emergency Care Journal 2024 volume 20:12790 That purplish huge hole on the chest wall Paola Rienzo,1 Erika Poggiali2 1Emergency Department, San Raffaele Hospital IRCCS, Milan; 2Emergency Medicine Unit, Fatebenefratelli Hospital, ASST Fatebenefratelli Sacco, Milan, Italy Question Given the patient history and the clinical presentation, what is the most likely diagnosis? 1. Pyogenic granuloma 2. Necrotizing fasciitis 3. Pyoderma gangrenosum 4. Martorell ulcer Answer The right answer is Pyoderma Gangrenosum (PG). PG is a rare ulcerative disorder that belongs to the category of neutrophilic der- matoses.1Despite its name, PG is not caused by infection or gan- grene. The etiology is unknown. In 70% of patients, PG is associ- ated with a systemic condition, including ulcerative colitis (5- 12%), Crohn's disease (1-2%), solid tumours, and haematologic malignancies (20%).2 The most common age of presentation is 30-50 years. Women are more commonly affected.1 The pathogen- esis is not fully understood.3 PG is a diagnosis of exclusion both clinically and histologically. There are no specific lab markers.1 A 54-year-old woman presented to our emergency department two months after the removal by Video-Assisted Thoracoscopic Surgery (VATS) of her middle lung lobe affected by severe inflammation and fibrosis. She was experiencing pain from a large ulcer (diameter 13 cm) starting from the surgical scar. She suffered from Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) syn- drome, and hypothyroidism. The laboratory results showed White Blood Cell count (WBC) 13,600/mm3 (N 76%), C- Reactive Protein (CRP) 12 mg/L (nv<6), and Erythrocyte Sedimentation Rate (ESR) 86 mm/h (nv<20). Repeat skin swabs were always negative. Skin biopsy documented granulocyte infil- tration in the dermis-hypodermis with tissue necrosis. For three weeks, the patient was treated with methylprednisolone 1 mg/Kg IV and colchicine 0.5 mg q12h orally. In the absence of a good response, oral cyclosporine 75 mg q12h was added. The ulcer gradually improved within a week. The pain was successfully treated with paracetamol 1 gr q8h IV. Correspondence: Erika Poggiali, Emergency Medicine Unit, Fatebenefratelli Hospital, ASST Fatebenefratelli Sacco, Milan, Italy. E-mail: poggiali.erika@gmail.com Key words: Pyoderma Gangrenosum, skin diseases, ulcer, skin lesions, thoracic surgery, VATS. Contributions: all the authors approved the final version and equally contributed to the work. Conflicts of interest: EP is a member of the editorial board of ECJ. This work was not supported by any grant. Availability of data and materials: all data underlying the findings are fully available upon reasonable request to the Corresponding Author. Ethics approval and consent to participate: as this was a descriptive case report and data was collected without patient identifiers, ethics approval was not required under our hospital’s Institutional Review Board guide- lines. Informed consent: the patient provided consent for access to medical records at the time of admission. Received: 5 July 2024. Accepted: 30 July 2024. Early view: 23 August 2024. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2024 Licensee PAGEPress, Italy Emergency Care Journal 2024; 20:12790 doi:10.4081/ecj.2024.12790 Publisher's note: all claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organiza- tions, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its man- ufacturer is not guaranteed or endorsed by the publisher. Non -co mmerc ial us e o nly Lesions are often initiated by minor trauma or scarring, and start with a small, red bump on the skin. Necrotic ulcers can develop rapidly over a period of 4-8 weeks. They usually exceed 10 cm in diameter and present with a purplish, undermined edge and sur- rounding erythema. Pathergy is the most important feature of PG. PG can affect any anatomical or surgical site. The skin over the tibia is a classic site. The head, oral cavity, and neck are rarely involved.4 The differential diagnosis includes mycobacterial and deep fungal infections. Martorell ulcers and necrotizing fasciitis must be always excluded.5 The treatment requires an interprofes- sional approach. The underlying systemic disease must be always treated.6 Wound care and pain control are key features in the treat- ment. Debridement must be performed very cautiously because of the association with pathergy.7 Systemic corticosteroids are the first-line therapy. Anti-TNF-alpha drugs such as etanercept and adalimumab have been used successfully.8 The prognosis is gener- ally good, but relapses are common. References 1. Schmieder SJ, Krishnamurthy K. Pyoderma Gangrenosum. Available from: https://www.ncbi.nlm.nih.gov/ books/NBK482223/ 2. Ashchyan HJ, Butler DC, Nelson CA, et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol. 2018;154:409-13. Erratum in: JAMA Dermatol. 2018;154:630. 3. Braswell SF, Kostopoulos TC, Ortega-Loayza AG. Pathophysiology of pyoderma gangrenosum (pg): an updated review. J Am Acad Dermatol. 2015;73:691-8. 4. Bissonnette C, Kauzman A, Mainville GN. Oral pyoderma gangrenosum: diagnosis, treatment and challenges: a systemat- ic review. Head Neck Pathol. 2017;11:427-41. 5. Vaysse-Vic M, Mathieu PA, Charissoux A, et al. Pyoderma gangrenosum or necrotising fasciitis? Diagnostic and thera- peutic wanderings. Orthop Traumatol Surg Res. 2017;103:615- 7. 6. Kaffenberger BH, Hinton A, Krishna SG. The impact of under- lying disease state on outcomes in patients with pyoderma gan- grenosum: a national survey. J Am Acad Dermatol. 2018;79:659-63.e2. 7. Pichler M, Larcher L, Holzer M, et al. Surgical treatment of pyoderma gangrenosum with negative pressure wound therapy and split thickness skin grafting under adequate immunosup- pression is a valuable treatment option: Case series of 15 patients. J Am Acad Dermatol. 2016;74:760-5. 8. Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recom- mendations for pyoderma gangrenosum: an evidence based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005;53:273-83. Images in emergency [Emergency Care Journal 2024; 20:12790] [page 75] Non -co mmerc ial us e o nly