Browsing 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
- 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.
- Caso dermatológico: líquen aureus segmentarPublication . Fernandes, I.; Carvalho, S.; Machado, S.; Alves, R.; Selores, M.ABSTRACT The authors describe a clinical case of a six-year-old boy with history of a segmental brownish maculopapular skin eruption on his left thoracic and lumbar wall, since the last four months. Based on clinical and histological findings he was diagnosed with segmental lichen aureus.
- Childhood Non-Langerhans Cell Histiocytosis – An Atypical CasePublication . Carvalho, S.; Machado, S.; Alves, R.; Vasconcelos, G.; Selores, M.Introduction: Histiocytoses correspond to a heterogeneous group of disorders characterized by the proliferation andinfiltration of histiocytes in tissues. For years, many of the histiocytoses where known by numerous names, reflecting the lack of understanding regarding their origin. Case report: A previously healthy, 4-month-old infant presented a cutaneous lesion of progressive growth on his nose since 1 month ago. The mother has a diagnosis of Sjögren syndrome. On examination, there was a firm well-defined erythematous plaque with 30 mm of diameter and raised edges, on the bridge of the nose. At the left cheek, left thoracic region and right arm there were also small erythematous papules with 5 days of evolution. The analytical study showed elevated antinuclear antibodies and anti-SS-A. Histopathological examination confirmed the diagnosis of non-Langerhans cells histiocytosis. Progressively, more annular erythematous papules and plaques appeared scattered over the face, trunk and limbs with spontaneous resolution of the first lesion on the nose. About 2 months after the initial evaluation, all lesions had regressed spontaneously, with normalization of analytical study at one year-old. Conclusion: Many authors believe that the different subtypes of histiocytosis represent only different stages of the same disease. Immunophenotyping of histiocytosis does not always identify the subtype involved, making the occurrence of atypical cases frequent.
- 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.
- 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.
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