SD - Artigos publicados em revistas indexadas na Medline/Pubmed
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Browsing SD - Artigos publicados em revistas indexadas na Medline/Pubmed 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.
- Brachioradial pruritus in a patient with cervical disc herniation and Parsonage-Turner syndromePublication . Carvalho, S.; Sanches, M.; Alves, R.; Selores, M.Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-year old woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900 mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.
- 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.
- Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas--unusual presentations of a common entityPublication . Pinto-Almeida, T.; Caetano, M.; Alves, R.; Selores, M.Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.
- Cutaneous necrobiotic xanthogranuloma (NXG)--successfully treated with low dose chlorambucil.Publication . Machado, S.; Alves, R.; Lima, M.; Leal, I.; Massa, A.Eur J Dermatol. 2001 Sep-Oct;11(5):458-62. Cutaneous necrobiotic xanthogranuloma (NXG)--successfully treated with low dose chlorambucil. Machado S, Alves R, Lima M, Leal I, Massa A. SourceService of Dermatology, Hospital Geral Santo António, Rua D. Manuel II, Edifício ex: Cicap, 4099-001 Porto, Portugal. basleite@esoterica.pt Abstract We report a case of necrobiotic xanthogranuloma in a 51 year-old white male patient presenting with a 6-year history of multiple indurated violaceous nodules and plaques involving the eyelids, trunk and extremities. He had an associated paraproteinemia (Ig G lambda), elevated sedimentation rate, cryoglobulinemia and hypocomplementemia. No extracutaneous involvement was detected. He was successfully treated with chlorambucil (2 mg/d for 7 months), leading to disappearance of all skin lesions.
- 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.
- 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