PT - Pele & Tegumentos
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Browsing PT - Pele & Tegumentos by Author "Alves, R."
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- 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.
- Ashy Dermatosis - Tratamento com ClofaziminaPublication . Pinto-Almeida, T.; Caetano, M.; Alves, R.; Selores, M.A Ashy dermatosis, ou erythema dyscromicum perstans, é uma dermatose rara de etiologia desconhecida. Os autores descrevem o caso de um homem de 46 anos saudável, com história de lesões cutâneas cinzentas assintomáticas no tronco com 1 ano de evolução. A constelação dos achados clínicos, histológicos e laboratoriais permitiu efectuar o diagnóstico de Ashy dermatosis. O doente iniciou tratamento com clofazimina oral, verificando-se resolução completa do quadro clínico e mantendo-se sem lesões cutâneas ao fim de 6 meses de vigilância. Estão descritas inúmeras opções terapêuticas para a Ashy dermatosis, no entanto nenhuma eficaz de forma consistente. O tratamento com clofazimina tem sido defendido devido ao seu efeito cosmético e a uma possível acção anti-inflamatória e imunomoduladora. Este caso documenta a eficácia terapêutica da clofazimina num doente com Ashy dermatosis
- Case for diagnosisPublication . Fernandes, I.; Sanches, M.; Alves, R.; Selores, M.Abstract We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.
- A dramatic case of Behçet disease successfully treated with infliximabPublication . Pinto-Almeida, T.; Amorim, I.; Alves, R.; Selores, M.Behçet disease is a chronic relapsing systemic disease with possible life-threatening presentations. Management of this disease can be challenging and reports of the off-label use of anti-TNFα agents for the treatment of severe manifestations are increasing, with good results. The authors report a case of Behçet disease with a sudden and severe multi-systemic onset successfully treated with infliximab.
- Effectiveness of Aprepitant in Patients with Refractory Pruritus Secondary to Sézary SyndromePublication . Fernandes, I.; Torres, T.; Selores, M.; Alves, R.; Lima, M.Background: In advanced stages, patients with Sézary Syndrome (SS) commonly report an ill-defined, severe and diffuse pruritus. Recently, it has been reported that Aprepitant, an oral neurokinin-1-receptor (NK1) antagonist, may have an important role in relief of refractory pruritus in patients with SS. Material and methods: A prospective study which included four patients with SS, in whom pruritus is the main symptom, was performed. Our purpose was to assess efficacy of Aprepitant for treatment of refractory pruritus, secondary to SS. Patients were treated with Aprepitant 80 mg/d during 10 days and then the dosage was reduced to alternate days. The length of treatment ranged between 4 and 23 weeks. Improvement was assessed by the Dermatology Life Quality Index (DLQI) questionnaire, which ranges from 0 to 30, with high scores indicating worse outcome and by Visual Analogue Scale (VAS) which varies from 0 to 10, with higher scores meaning severe pruritus. Results: Prior to treatment, subjects had severe pruritus with mean DLQI score of 21.5 (SD ± 2.4) and mean VAS score of 9.0 (SD ± 0.8). At the end of the treatment, a statistically significant reduction in both indexes (p<0.05) was evident. In all patients, an improvement of pruritus was rapidly observed after the first week of therapy. No side effects were reported. Conclusion: The study confirms the effectiveness and safety of Aprepitant as an antipruritic agent in patients with refractory pruritus secondary to SS.
- Eritema multiforme major associado à sertralinaPublication . Carvalho, S.; Sanches, M.; Velho, G.; Alves, R.; Selores, M.A sertralina é frequentemente utilizada para o tratamento de síndromes depressivos e ansiosos. Os efeitos secundários são geralmente transitórios e dependentes da dosagem. Descrevemos o caso clínico de uma mulher de 76 anos com síndrome depressivo e introdução recente da sertralina que recorreu ao serviço de urgência por febre e erupção cutânea difusa com dois dias de evolução. Ao exame objetivo observavam-se pápulas eritematosas em alvo dispersas pelo corpo e exulcerações da mucosa oral. Foi efetuada uma biopsia cutânea para exame histopatológico com diagnóstico de eritema multiforme major secundário à sertralina. A doente suspendeu o fármaco em causa e iniciou prednisolona oral com resolução do quadro clínico. Embora pouco frequentes, existem casos descritos de reação cutânea grave associada à sertralina.
- 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.
- Exuberant cutaneous ulcers on the buttocks caused by multi-resistantPublication . Pinto-Almeida, T.; Rosmaninho, A.; Lobo, I.; Alves, R.; Selores, M.Cutaneous infection develops because of environmental and local factors, host immunity, and organism adherence and virulence. The authors report a case of exuberant cutaneous ulcers on the buttocks of a diabetic patient. Microbiologic examination allowed the identification of Klebsiella pneumoniae and complete resolution was achieved with the appropriate antibiotic
- Giant cutaneous horn on the lower lipPublication . Pinto-Almeida, T.; Oliveira, A.; Velho, G.; Alves, R.; Caetano, M.; Selores, M.Cutaneous horn is a conical hyperkeratotic projection of the skin composed of compact keratin. A wide range of pathologic conditions may be found at its base, including a significant proportion of malignant tumors. A notable, giant cutaneous horn uncovering a keratoacanthoma/well-differentiated squamous cell carcinoma is presented, highlighting the importance of histopathological examination to rule out malignancy because clinical features cannot assure a correct diagnosis.
- Penile Kaposi sarcoma: A case of complete resolution with highly active antiretroviral therapy alonePublication . Pinto-Almeida, T.; Torres, T.; Rosmaninho, A.; Sanches, M.; Alves, R.; Caetano, M.; Selores, M.Kaposi sarcoma remains an important cause of morbidity in HIV-infected patients. Regardless of the recent pharmacological progress, treatment of this malignancy is still disappointing. We report the case of a patient with Kaposi sarcoma in an unusual localization, the penis, which completely resolved with highly active antiretroviral therapy alone