Hrev_master Question Given the patient’s history, what is the most likely diagnosis? 1. Erythema nodosum 2. Paraneoplastic pemphigus 3. Fungal skin infection 4. Urticarial vasculitis Emergency Care Journal 2022; volume 18:10498 Correspondence: Erika Poggiali, Emergency Department, “Guglielmo da Saliceto” Hospital, Via Giuseppe Taverna 49, Piacenza, Italy. Tel.: +39.0523.303044 E-mail: poggiali.erika@gmail.com Key words: Skin lesions; paraneoplastic pemphigus; haematology. Contributions: EP and JP collected details of the case and drafted the manuscript. GO and JP cared for the patient. All authors approved the final version and stated the integrity of the whole work. Conflicts of interest: The authors declare no conflict of interest. EP is member of the editorial board of Emergency Care Journal. Availability of data and materials: All data underlying the findings are fully available upon reasonable request to Erika Poggiali, E.Poggiali@ausl.pc.it 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 guidelines. Informed consent: The patient provided consent for the access to med- ical records at the time of admission. Received for publication: 5 April 2022. Revision received: 21 April 2022. Accepted for publication: 21 April 2022. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2022 Licensee PAGEPress, Italy Emergency Care Journal 2022; 18:10498 doi:10.4081/ecj.2022.10498 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. [Emergency Care Journal 2022; 18:10498] [page 1] Anaemia, thrombocytopenia and skin lesions Erika Poggiali,1 Giorgio Orofino,2 Jacopo Peccatori2 1Emergency Department, Guglielmo da Saliceto Hospital, Piacenza; 2Unit of Hematology and Bone Marrow Transplantation, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy Descriptive legend. A 73-year-old man affected by hyperuricemia, dyslipidaemia and hypothyroidism presented to the emergency room with a 3-month history of fever, exertional dyspnea, pro- gressive asthenia, and painless not itchy skin lesions. Physical exam showed purplish papules and plaques affecting any area of his body, and a slight bilateral oedema of his legs. Laboratory studies revealed a severe macrocytic anaemia (haemoglobin 4.8 g/dL, mean cell volume 119 fL) and thrombocytopenia (34,000/mm3) with hyperferritinemia (1894 ng/mL, normal value <400) and increased serum B12 (1412 pg/mL, normal value 197-771), associated with ESR 71 mm/h (normal value 1-15), CRP 139 mg/L (normal value <6), and procalcitonin 1.05 ng/mL (normal value <0.5). Non -co mmerc ial us e o nly Answer Paraneoplastic Pemphigus (PNP) is the most likely. The patient was investigated with a colonoscopy and an upper endoscopy, that excluded a gastrointestinal bleeding and neoplasms. PET did not detect any occult malignancies. A bone marrow biopsy was per- formed, and a diagnosis of Refractory Anaemia with Excess Blasts type 2 (RAEB-2) was done. A punch biopsy confirmed the diagno- sis of PNP. PNP showed a good response to the targeted therapy for RAEB-2, but not a complete resolution. PNP is a rare life-threatening mucocutaneous autoimmune dis- ease associated with malignancies,1 the most frequent of which include lymphoma, leukaemia, and Castleman’s disease.2,3 Since some cases of PNP were diagnosed before an underlying malig- nancy was detected, PNP can be considered as a marker for occult malignancy. PNP manifests as polymorphic mucocutaneous erup- tions, ranging from blisters and erosions to lichenoid eruptions, as reported in our case, to onychodystrophy and alopecia.4 A single patient can present different types of lesions. Some patients suffer from dyspnea due to bronchiolitis obliterans as extracutaneous manifestation of PNP, more common in Castleman’s disease.5 The pathogenetic mechanism is not completely known. PNP is charac- terized by the production of autoantibodies against the plakin fam- ily proteins, which are target antigens of ordinary pemphigus. The course of PNP is not correlated with that of the associated malig- nancy, and the prognosis is generally poor, with high mortality rate due to sepsis or multi-organ failure. There are no consistently effective treatments. Rituximab, intravenous immunoglobulins, and plasmapheresis have shown promising effects.6 Bronchiolitis obliterans is resistant to therapy, and lung transplantation is the last therapeutic option for respiratory failure.7 References 1. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphi- gus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990;323:1729-35. 2. Ohzono A, Sogame R, Li X, et al. Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. Br J Dermatol 2015;173:1447–52. 3. Lim JM, Lee SE, Seo J, et al. Paraneoplastic pemphigus asso- ciated with a malignant thymoma: a case of persistent and refractory oral ulcerations following thymectomy. Ann Dermatol 2017;29:219–22. 4. Lee SE, Kim SC. Paraneoplastic pemphigus. Dermatol Sinica 2010;28:1–14. 5. Lee J, Bloom R, Amber KT. A systematic review of patients with mucocutaneous and respiratory complications in parane- oplastic autoimmune multiorgan syndrome: castleman’s dis- ease is the predominant malignancy. Lung 2015;193:593–6. 6. Kim JH, Kim SC. Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa. Front Immunol 2019;10:1259. 7. Chin AC, Stich D, White FV, et al. Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: a rare case of Castleman’s disease with respiratory fail- ure requiring lung transplantation. J Pediatr Surg 2001;36:E22. Images in Emergency [page 2] [Emergency Care Journal 2022; 18:10498] Non -co mmerc ial us e o nly