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- Biologic therapy for psoriasis - still searching for the best target.Publication . Pinto-Almeida, T.; Torres, T.Psoriasis is a chronic skin disease that results from the complex interaction between genetic and environmental factors. Over the last few decades, scientific evidence has redirected the focus of therapeutic studies to the immunologic pathways underlying its pathogenesis. This led to the biologic boom that we are currently experiencing, with the development and approval of targeted progressively more selective biological therapies and ongoing clinical trials of increasingly specific drugs, given their important implications for long-term efficacy and safety. Nevertheless, the search for the optimal biologic is still ongoing, and the best target has yet to be found.
- Adult mastocytosis: a review of the Santo António Hospital 's experience and an evaluation of World Health Organization criteria for the diagnosis of systemic diseasePublication . Fernandes, I.; Teixeira, M.; Freitas, I.; Selores, M.; Alves, R.; Lima, M.BACKGROUND: Mastocytosis is a clonal disorder characterized by the accumulation of abnormal mast cells in the skin and/or in extracutaneous organs. OBJECTIVES: To present all cases of mastocytosis seen in the Porto Hospital Center and evaluate the performance of World Health Organization diagnostic criteria for systemic disease. METHODS: The cases of twenty-four adult patients with mastocytosis were reviewed. Their clinical and laboratorial characteristics were assessed, and the properties of the criteria used to diagnose systemic mastocytosis were evaluated. RESULTS: The age of disease onset ranged from 2 to 75 years. Twenty-three patients had cutaneous involvement and 75% were referred by dermatologists. Urticaria pigmentosa was the most common manifestation of the disease. One patient with severe systemic mast cell mediator-related symptoms showed the activating V560G KIT mutation. The bone marrow was examined in 79% of patients, and mast cell immunophenotyping was performed in 67% of the participants. Systemic disease was detected in 84% of cases, and 81% of the sample had elevated serum tryptase levels. All the diagnostic criteria for systemic mastocytosis had high specificity and positive predictive value. Bone marrow biopsy had the lowest sensitivity, negative predictive value and efficiency, while the highest such values were observed for mast cell immunophenotyping. Patients were treated with regimens including antihistamines, sodium cromoglycate, alpha-interferon, hydroxyurea and phototherapy. CONCLUSIONS: Cutaneous involvement is often seen in adult mastocytosis patients, with most individuals presenting with indolent systemic disease. Although serum tryptase levels are a good indicator of mast cell burden, bone marrow biopsy should also be performed in patients with normal serum tryptase, with flow cytometry being the most adequate method to diagnose systemic disease.
- Interleucina-17 como Alvo Terapêutico na PsoríasePublication . Torres, T.; Filipe, P.A psoríase é uma doença crónica, inflamatória, imunomediada que afecta cerca de 1-3% da população geral. O avanço no conhecimento da patogénese da psoríase levou ao desenvolvimento de novos fármacos direccionados para vias imunitárias mais específicas que os existentes. A IL-17 e os linfócitos Th17 têm um importante papel na patogénese de várias doenças auto-imunes e imunomediadas, incluindo a psoríase. A IL-17A é uma citocina pró-inflamatória, produzida pelos linfócitos Th17 juntamente com outras citocinas efetoras, como a IL-17F e IL-22. Esta citocina é igualmente expressa por outras células do sistema imune inato, incluíndo os mastócitos, neutrófilos e células dendríticas, todas elas encontradas nas lesões de psoríase. Por esta razão a IL-17 surgiu como um alvo terapêutico potencial. Vários agentes que inibem a IL-17 estão em desenvolvimento e os resultados clínicos preliminares são bastante promissores, confirmando a importância da IL-17 na fisiopatologia da psoríase. A intervenção selectiva no sistema imune desta nova classe de agentes biotecnológicos, torna-os uma abordagem terapêutica interessante no controlo de doenças auto-imunes e imunomediadas, como na psoríase. Num futuro próximo, estas novas terapêuticas poderão constituir uma alternativa válida aos agentes biológicos actualmente disponíveis.
- Psoriasis and Metabolic SyndromePublication . Sales, R.; Torres, T.Psoriasis is a chronic, systemic inflammatory disease associated with several cardiometabolic comorbidities, such as obesity, insulin resistance, dyslipidemia, and hypertension, and with clinically significant increased risk of cardiovascular disease and cardiovascular mortality. These comorbidities are components of the metabolic syndrome. Multiple epidemiologic studies have revealed a high prevalence of metabolic syndrome in patients with psoriasis compared with other skin diseases. Genetic susceptibility and overlapping inflammatory pathways may be potential biologic links underlying this association. Understanding the interrelationship between these conditions is important for the management of psoriasis and its associated comorbidities. This review will focus on the range of these comorbidities, with emphasis on the metabolic syndrome, aiming to encourage physicians to screen patients with psoriasis for cardiometabolic disorders and risk factors
- Eruptive syringomasPublication . Teixeira, M.; Ferreira, M.; Machado, S.; Alves, R.; Selores, M.Eruptive syringoma is a rare clinical presentation of a benign tumor of the eccrine ducts. It consists in successive crops of small skin-colored papules on the anterior body surfaces. It generally occurs in the peri-pubertal period. Treatment of this benign condition is cosmetic only. A case of a 19-year-old female with a 5-year history of eruptive syringoma is presented.
- Perioral pigmentation: What is your diagnosis?Publication . Santos, P.; Neto, C.; Machado, S.; Lobo, I.; Soares, J.; Selores, M.Pigmented spots in the skin and mucosa (lentigines) can be found in various diseases called familial lentiginosis syndromes; Peutz-Jeghers syndrome (PJS) is one of them. It is characterized by the association of mucocutaneous melanin pigmentation and hamartomatous gastrointestinal polyps. Patients with PJS are at increased risk of intussusception and cancer development (gastrointestinal and non-gastrointestinal tumors). We present a 5-year-old girl with pigmented macules of perioral and perinasal skin, lips, and buccal mucosa and review lentiginoses and the surveillance of PJS.
- Sezary Syndrome presenting with leonine facies and treated with low-dose subcutaneous alemtuzumabPublication . Oliveira, A.; Lobo, I.; Alves, R.; Lima, M.; Selores, M.Cutaneous T-cell lymphomas (CTCL) comprise a group of diseases characterized by the accumulation of malignant T cells within the skin. Sezary syndrome represents an aggressive form of CTCL, in which the skin is diffusely affected and the peripheral blood is involved. It is characterized by the triad of generalized erythroderma, lymphadenopathy, and neoplastic T cells (Sezary cells) in the skin, lymph nodes, and peripheral blood. Leonine facies is rare and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face. It can occur in cutaneous T-cell lymphomas that progress during years without therapy. We present the case of a 54-year-old man with Sezary syndrome presenting with leonine facies, unresponsive to conventional therapies; he exhibited a promising response to subcutaneous low-dose alemtuzumab.
- Transient peripheral facial nerve paralysis after local anesthetic procedurePublication . Rosmaninho, A.; Lobo, I.; Caetano, M.; Taipa, R.; Magalhães, M.; Costa, V.; Selores, M.Complications may arise after laser therapy of the face. The most common ones are bleeding and infections; facial nerve paresis or paralysis is rarely reported. We describe a case of a transient peripheral facial nerve paralysis after laser therapy of an epidermal verrucous nevus localized at the left preauricular area.
- Primary cutaneous marginal zone B cell lymphoma of the face: A challenging diagnosisPublication . Oliveira, A.; Caetano, M.; Alves, R.; Lima, M.; Selores, M.The primary cutaneous marginal zone B cell lymphoma is a small B cell lymphoma, including cells of the marginal zone, lymphoplasmacytic cells, and plasma cells. Clinically it manifests as erythematous or erythematous-violaceous papules, plaques, or nodules, single or multiple, most often located to the extremities. Its course is usually indolent, with a survival at 5 years of approximately 97 percent. The tumor exhibits a tendency towards local recurrence, but spread to locations outside the skin is extremely rare. We present a case report of a man, 80 years of age, with a primary cutaneous marginal B cell lymphoma of the chin, an atypical location.
- Dermatology inpatient consultation in a Portuguese university hospitalPublication . Fernandes, I.; Velho, G.; Selores, M.BACKGROUND: Cutaneous findings are frequent in hospitalized patients. There are few reports regarding this subject. OBJECTIVES: To identify the frequency and the impact on clinical courses of dermatologic conditions in patients in the inpatient setting and compare the data with other similar studies. METHODS: Retrospective review of 274 hospitalized patients in non-dermatology inpatient departments who were observed by a dermatology consultant in a Portuguese central university hospital during a year. RESULTS: A total of 282 consultations were performed. The services requesting consultation most frequently were internal medicine (33.7%), surgery (10.3%), and pediatrics (8.9%). Skin infections (33.2%), eczemas (9.5%), and drug eruptions (7.3%) were the most common diagnoses. Admission diagnosis was modified in 9 cases (3.3%) by the dermatology consultant. CONCLUSION: Dermatoses are frequently misdiagnosed by non-dermatologists. Common skin diseases were responsible for most of dermatology inpatient consultations. However, in some cases the dermatology consultation changed the primary main diagnosis and had an important impact on the clinical course.