[Correction to the Article "Hansen's Disease: An Unusual Manifestation of an Ancient Disease", Published on Acta Med Port 2024 Mar;37(3):215-219]. / Errata ao Artigo "Doença de Hansen: Apresentação Incomum de uma Doença Antiga", Publicado em Acta Med Port 2024 Mar;37(3):215-219.

On page 215, list of authors, where it reads (in red): Mário FERREIRA1, Carlos GRIJÓ2, Joana PAULO1, Marta FONSECA1, Zélia NEVES1 It should read (in bold): Mário FERREIRA1, Carlos GRIJÓ2, Joana PAULO1, Marta FONSECA1, Zélia NEVES1, Rita BOUCEIRO MENDES3, Pedro VASCONCELOS3 On the same page 215, footer (authors affiliation), where it reads (in red): 1. Medicina III. Hospital Fernando Fonseca. Amadora. Portugal. 2. Serviço de Medicina Interna. Centro Hospitalar Universitário de São João. Porto. Portugal. It should read (in bold): 1. Medicina III. Hospital Fernando Fonseca. Amadora. Portugal. 2. Serviço de Medicina Interna. Centro Hospitalar Universitário de São João. Porto. Portugal. 3. Serviço de Dermatologia. Hospital de Santa Maria. Centro Hospitalar Universitário Lisboa Norte. Lisboa. Portugal. Article published with errors: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/20599.

Na página 215, na linha de autoria onde se lê, (a vermelho): Mário FERREIRA1, Carlos GRIJÓ2, Joana PAULO1, Marta FONSECA1, Zélia NEVES1 Deverá ler-se (a negrito): Mário FERREIRA1, Carlos GRIJÓ2, Joana PAULO1, Marta FONSECA1, Zélia NEVES1, Rita BOUCEIRO MENDES3, Pedro VASCONCELOS3 Na mesma página 215, em rodapé (afiliação dos autores), onde se lê (a vermelho): 1. Medicina III. Hospital Fernando Fonseca. Amadora. Portugal. 2. Serviço de Medicina Interna. Centro Hospitalar Universitário de São João. Porto. Portugal. Deverá ler-se (a negrito): 1. Medicina III. Hospital Fernando Fonseca. Amadora. Portugal. 2. Serviço de Medicina Interna. Centro Hospitalar Universitário de São João. Porto. Portugal. 3. Serviço de Dermatologia. Hospital de Santa Maria. Centro Hospitalar Universitário Lisboa Norte. Lisboa. Portugal. Artigo publicado com erros: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/20599.

T-large Granular Lymphocytic Leukemia: A Rare Diagnosis in a Young Woman With Fever, Necrotic Skin Lesions and Cytopenias.

T-large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative disorder. The diagnosis is established by identifying an abnormally high number of clonal granular T lymphocytes in the peripheral blood and eventually in the bone marrow, in cases with medullary infiltration. The majority of patients present with symptoms related to neutropenia and this condition may be associated with autoimmune diseases in up to a third of cases. The authors describe the case of a 26-year-old patient admitted with subacute high fever and bullous dermatitis with necrotic lesions with central bullae. Analytically, she presented anemia and leukopenia with severe neutropenia of 200 cells/L. Skin lesions were compatible with ecthyma and the skin biopsy revealed aspects compatible with leukocytoclastic vasculitis. The myelogram and bone biopsy revealed hypoplasia of the myeloid line and a pathological T population of CD8+, TIA-1+ and granzyme B+, which were associated with compatible flow cytometry (CD3+, T-cell receptor (TCR) Alpha-Beta+, CD5+, CD2+, with loss of CD7 antigen expression) established the diagnosis of T-LGLL. The patient had a favorable evolution, with cytopenias almost returning to normal after two months. She began follow-up at a Hematology Reference Center, remaining asymptomatic without specific treatment considering the indolent course of the disease.

[Hansen's Disease: An Unusual Manifestation of an Ancient Disease]. / Doença de Hansen: Apresentação Incomum de uma Doença Antiga.

Hansen's disease, commonly known as leprosy, is an infectious disease caused by Mycobacterium leprae. Being rare in developed countries, it is an increasingly common imported disease due to the migratory flow from countries where it is endemic. We present the case of a 21-year-old man who went to the emergency department with complaints of additive polyarthralgia involving large joints, papules, and erythematous plaques on the limbs with bullae and central necrosis and fever with chills for one week. Skin biopsy was performed revealing neutrophilic infiltrate with perineural granulomas. The bacilloscopy detected acid-alcohol resistant bacilli. The diagnosis of multibacillary HD with type 2 lepromatous reaction (erythema nodosum leprosum - ENL) was established, showing clinical improvement under corticosteroid therapy. ENL usually presents with painful lesions, being an atypical presentation of leprosy, especially in the presence of bullae and necrosis, making diagnosis difficult and challenging. Social stigma is often present making it difficult to accept the disease as well as adherence to treatment.

A doença de Hansen, vulgarmente conhecida como lepra, é uma doença infecciosa causada por Mycobacterium leprae. Sendo rara nos países desenvolvidos, configura uma doença de importação cada vez mais frequente considerando o fluxo migratório de países onde é endémica. Apresentamos o caso de um homem de 21 anos que recorreu ao serviço de urgência por poliartralgias de caráter aditivo envolvendo grandes articulações, pápulas e placas eritematosas nos membros com bolhas e necrose central e febre com calafrio com uma semana de evolução. Foi realizada biópsia cutânea que revelou infiltrado neutrofílico com granulomas de distribuição perineural e baciloscopia com deteção de bacilos ácido-álcool resistentes. Foi estabelecido o diagnóstico de DH multibacilar com reação lepromatosa tipo 2 (eritema nodoso leproso), apresentando melhoria clínica sob corticoterapia. O eritema nodoso leproso cursa habitualmente com lesões dolorosas, configurando uma apresentação atípica de lepra, sobretudo na presença de bolhas e necrose, tornando este diagnóstico altamente desafiante. O estigma social é frequentemente limitativo na aceitação da doença e adesão ao tratamento.

A Refractory Case of Amiodarone Thyrotoxicosis.

Amiodarone is frequently used to control cardiac arrhythmias, like atrial fibrillation. Despite its benefits, it has many adverse effects, particularly on the thyroid gland. We describe the case of a patient treated with amiodarone for paroxysmal atrial fibrillation, admitted to the emergency room with atrial fibrillation with a rapid ventricular response. Type II thyrotoxicosis was identified as the cause of the refractory arrhythmia. Since its refractoriness to both pharmacological and electrical therapy, there was a need to proceed with plasmapheresis and total thyroidectomy for hormonal and cardiac rhythm control. Therefore, it is essential to monitor the toxicity of amiodarone, a drug that can have both beneficial and devastating effects.

Pacemaker lead as an iatrogenic cause of right heart failure: Case report. / Electrocatéter como causa iatrogénica de insuficiência cardíaca direita ­ relato de um caso.

Technical advances in health care have improved patient survival and quality of life, but are not devoid of complications. We present the case of a 74-year-old woman with a history of hypertensive heart disease with preserved systolic function, atrial fibrillation and dyslipidemia. She had a DDDR pacemaker implanted in 2005 due to symptomatic complete atrioventricular block. The patient reported progressive fatigue, weakness, ascites with abdominal discomfort, and lower limb edema, accompanied by non-specific hepatic cholestasis on biochemical testing. Abdominal ultrasound revealed homogeneous hepatomegaly and dilatation of the inferior vena cava and upper hepatic veins, suggestive of congestive hepatopathy. Echocardiography revealed tricuspid regurgitation progressively worsening over the previous four years and dilatation and progressive dysfunction of the right ventricle, with preserved left ventricular function. The transesophageal echocardiogram revealed severe tricuspid regurgitation with flail septal leaflet and marked dilatation of the tricuspid annulus due to mechanical interference of the pacemaker lead, which was adhering to the septal leaflet. Minimally invasive surgical treatment was performed with partial resection of the leaflet, placement of a tricuspid annuloplasty ring and replacement of the pacemaker lead. Regression of the congestive symptoms was observed, and the postoperative echocardiogram showed the tricuspid annuloplasty ring with no evidence of stenosis and only slightly dilated right chambers with moderate pulmonary hypertension. Six months after the procedure, the patient suffered an acute neurological event and died.

Relapsing klebsiella pneumoniae liver abscesses in a non-asian diabetic man / Abcessos hepáticos recidivantes provocados por klebsiella pneumoniae num homem diabético não asiático

The Klebsiella pneumoniae invasive syndrome, first described in Asia, is being reported in other parts of the world. It causes liver abcsesses, with or without extrahepatic lesions. Diabetes mellitus is the most common hosts' underlying condition. It's frequent among asian people, even outside Asia, appearing to exist genetic factors, not yet established, increasing the risk. We present a case of a 55-year-old portuguese white diabetic man, who had a previous hospital admittion due to Klebsiella penumoniae pneumonia and a two-week latter diagnosis of liver abcsess. 4 years latter he was readmitted with nausea, chills, fever and myalgias. He had elevated inflammatory markers and the CT-scan showed 2 liver abscesses. Klebsiella pneumoniae was isolated in blood and antibiotics were given with complete clinical and imaging resolution. We present this case of relapsing Klebsiella pneumonia liver abscesses in a non-asian man emphasizing the growing incidence of this condition in Europe.

O síndrome invasivo provocado por Klebsiella pneumoniae, descrito pela primeira vez na Ásia, tem vindo a ser reportado em outras partes do mundo. Provoca abcessos hepáticos, com ou sem lesões extra-hepáticas associadas. A diabetes mellitus é a condição predisponente do hospedeiro mais comum. É frequente nos indivíduos asiáticos, mesmo fora da Ásia, parecendo existir factores genéticos, ainda não estabelecidos, que aumentam o risco da infecção. Apresentamos o caso de um homem português de 55 anos, diabético que tinha uma admissão prévia no hospital por pneumonia provocada por Klebsiella pneumoniae, com diagnóstico de abcesso hepático 2 semanas depois. 4 anos mais tarde, foi readmitido com um quadro clínico de nauseas, calafrios, febre e mialgias. Apresentava parâmetros inflamatórios elevados e a tomografia computorizada (TC) abdominal revelou 2 abcessos. Foi isolada Klebsiella pneumoniae em hemoculturas e foram administrados antibióticos com completa resolução clínica e imagiológica. Apresentamos este caso de abcessos hepáticos recidivantes por Klebsiella pneumoniae num homem não asiático, enfatizando a incidência crescente desta entidade na Europa.

Abcesso cerebral num doente com Rendu-Osler-Weber / Cerebral abscess in a patient with Rendu-Osler-Weber

A telangiectasia hemorrágica hereditária é uma doença familiar rara, descrita pela primeira vez no final do século 19, caracterizada por telangiectasias mucocutâneas e viscerais, e por malformações arteriovenosas. Manifestações neurológicas ocorrem em 8 a 12% dos doentes e resultam em mais de metade dos casos de malformações arteriovenosas pulmonares. A telangiectasia hemorrágica hereditária é uma das causas mais frequentes de abcessos cerebrais recorrentes, múltiplos e bilaterais, tendendo estes a ocorrerem na 3a para a 5a década. Apresentamos o caso de uma mulher de 88 anos admitida por prostração, febre e insuficiência respiratória, com a suspeita de metastização pulmonar de neoplasia oculta, cuja investigação revelou, entretanto, um abcesso cerebral secundário a malformações arteriovenosas pulmonares. Apesar da antibioterapia e da drenagem cirúrgica do abcesso, a paciente veio a falecer. O prognóstico após drenagem dos abcessos sem ressecção das lesões pulmonares é desfavorável, podendo sua elevada mortalidade refletir um atraso diagnóstico pela falta de reconhecimento dessa associação.

Hereditary hemorrhagic telangiectasia is a rare familial disorder, first described in the late 19th century characterized by multiple mucocutaneous and visceral telangiectases and arteriovenous malformations. Neurologic manifestations occur in 8 to 12% of patients and result from pulmonary arteriovenous malformations in 50% of cases. Hereditary hemorrhagic telangiectasia is one of the most frequent causes of recurrent, multiple and bilateral cerebral abscess and these tend to occur in the 3rd to 5th decade. We present the case of an 88-year-old woman admitted for prostration, fever and respiratory failure who was thought to have pulmonary metastasis of an unknown tumor but investigation revealed a brain abscess secondary to pulmonary arteriovenous malformations. Despite antibiotics and surgical drainage the patient died. The prognosis of brain abscess drainage without resection of the pulmonary lesions is not favorable. The high mortality of brain abscess in these patients may reflect a delay in diagnosis due to lack of recognition of this association.

Panniculitis - A Rare Manifestation of Acute Pancreatitis.

Pancreatic panniculitis is a rare skin disorder that occurs in 2-3% of pancreatic diseases, mostly associated with acute or chronic pancreatitis. Its pathophysiology is still unclear, but the release of pancreatic enzymes in circulation can be responsible for this disorder. The typical histological features are adipocyte necrosis with neutrophils infiltrate and typical "ghost cells". Its treatment, clinical course and prognosis are usually related with the pancreatic disease. We present a case of a 39-year-old man who presented with fever, abdominal pain, nausea and vomiting after a copious alcohol ingestion. Acute alcohol-induced pancreatitis was diagnosed. 9 days after admission he developed tender erythematous subcutaneous nodules, whose biopsy was consistent with pancreatic panniculitis. There was clinical and laboratory improvement with supportive treatment as well as skin nodules regression. Although pancreatic panniculitis is a rare condition, physicians must be aware of it to avoid delay in the diagnosis.

A paniculite pancreática é uma condição cutânea rara que ocorre em 2­3% das doenças pancreáticas, estando sobretudo associada às pancreatites aguda e crónica. A sua fisiopatologia ainda é incerta, mas a libertação de enzimas pancreáticas na circulação parece estar implicada. Os aspectos histológicos característicos consistem em necrose dos adipócitos com infiltrado neutrofílico e as típicas "ghost cells". O seu tratamento, evolução clínica e prognóstico estão habitualmente relacionados com a doença pancreática.Apresentamos o caso de um homem de 39 anos com um quadro de febre, dor abdominal, náuseas e vómitos após uma ingestão copiosa de álcool. Foi diagnosticada uma pancreatite aguda alcoólica. 9 dias após a admissão, surgiram nódulos eritematosos subcutâneos dolorosos, cuja biópsia foi consistente com paniculite pancreática. Houve melhoria clinica e laboratorial com o tratamento de suporte, assim como regressão dos nódulos cutâneos.Embora a paniculite pancreática seja uma condição rara, os médicos devem tê-la presente para evitar o atraso no diagnóstico.