Browsing by Author "Vieira, AP"
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- Alopécia Areata: Análise Retrospectiva da Consulta de Dermatologia. Pediátrica (2000-2008)Publication . Rocha, J; Ventura, F; Vieira, AP; Pinheiro, AR; Fernandes, F; Brito, CINTRODUCTION: Alopecia areata usually presents as patchy, nonscarring hair loss. It seems to be an immune mediated disease, whereas genetic predisposition, environmental and psychological triggers may be involved in its aetiology. OBJECTIVES: To study the epidemiology, clinical aspects, associations, and treatment of alopecia areata in the paediatric population of Peadiatric Dermatology outpatients over a 9-year period. Some psychologic characteristics were also assessed. METHODS: Descriptive and retrospective study of all newly diagnosed AA cases seen from January 2000 to December 2008 at the Hospital de São Marcos' Paediatric Dermatology Department. Fifteen patients with AA were interviewed for psycologic evaluation. RESULTS: Forty-eight cases (54% male/46% female) were identified. Mean age at presentation was 7.8 years. Family history of AA was reported in 10% of the cases, and in 25% there was a personal and/or family history of atopy. The majority of patients (82%) had mild disease and topical corticotherapy was the first-line treatment for limited AA. Fifty-four percent of these patients had a complete resolution of the lesions with treatment. Systemic treatment (corticosteroids and/or ciclosporin) was used in 71% of patients with extensive disease (more than 50% hair loss). Only one of these patients had a sustained clinical improvement after treatment. Twelve out of 15 respondents (80%) recalled stressful events preceding hair loss. DISCUSSION: Our findings are similar to those reported in other studies. Epidemiologic studies of AA are available in adulthood but there is a paucity of literature on children with AA. A holistic approach is important in the management of childhood AA as the disease can have a severe psychologic impact on an individual's well-being.
- Cannabis arteritis: ever more important to considerPublication . Santos, RP; Resende, C; Vieira, AP; Brito, CCannabis arteritis (CA) is a major and underdiagnosed cause of peripheral arterial disease in young patients. A 34-year-old man, daily smoker of 20 cigarettes and two cannabis cigarettes for 14 years, presented with a necrotic plaque of left hallux for 3 weeks. The Doppler ultrasound and angiography were compatible with severe Buerger's disease. Submitted to a revascularisation procedure and hypocoagulation with rivaroxaban. He had ceased smoking but maintained consumption of cannabis. Owing to the persistence of distal necrosis, amputation of the hallux was performed with good evolution. CA is a subtype of Buerger's disease. It is poorly known but increasingly prevalent and manifests in cannabis users regardless of tobacco use. The drug is considered at least a cofactor of the arteriopathy. The most effective treatment is cessation of consumption. Being cannabis one of the most consumed drugs, its mandatory to ask about its use in all young patients with arteriopathy.
- A Case of IFAP Syndrome with Severe Atopic DermatitisPublication . Araújo, C; Gonçalves-Rocha, M; Resende, C; Vieira, AP; Brito, CIntroduction. The IFAP syndrome is a rare X-linked genetic disorder characterized by the triad of follicular ichthyosis, atrichia, and photophobia. Case Report. A three-month-old Caucasian, male patient was observed with noncicatricial universal alopecia and persistent eczema from birth. He had dystrophic nails, spiky follicular hyperkeratosis, and photophobia which became apparent at the first year of life. Short stature and psychomotor developmental delay were also noticed. Histopathological examination of skin biopsy on left thigh showed epidermis with irregular acanthosis, lamellar orthokeratotic hyperkeratosis, and hair follicles fulfilled by parakeratotic hyperkeratosis. The chromosomal study showed a karyotype 46, XY. Total IgE was 374 IU/mL. One missense mutation c.1360G>C (p.Ala454Pro) in hemizygosity was detected on the MBTPS2 gene thus confirming the diagnosis of IFAP syndrome. Conclusions. We describe a boy with a typical clinical presentation of IFAP syndrome and severe atopic manifestations. A novel missense mutation c.1360G>C (p.Ala454Pro) in MBTPS2 gene was observed. The phenotypic expression of disease is quantitatively related to a reduced function of a key cellular regulatory system affecting cholesterol and endoplasmic reticulum homeostasis. It can cause epithelial disturbance with failure in differentiation of epidermal structures and abnormal skin permeability barrier. However, no correlation phenotype/genotype could be established.
- Cutaneous polyarteritis nodosa in a child following hepatitis B vaccinationPublication . Ventura, F; Antunes, H; Brito, C; Pardal, F; Pereira, T; Vieira, AP
- Cutis marmorata telangiectatica congenitalPublication . Resende, CI; Araújo, C; Vieira, AP; Brito, C
- Cyclosporin A treatment in severe childhood psoriasisPublication . Pereira, TM; Vieira, AP; Fernandes, JC; Sousa-Basto, AThough used occasionally, systemic therapies in severe childhood psoriasis have not been systematically investigated. Cyclosporin A (CysA) is effective in adults with severe psoriasis but there are no extensive data regarding the efficacy and safety of its use in childhood psoriasis. In this paper, we describe six children aged between 11 months and 13 years (average: 7.6 years) treated with CysA microemulsion formulation for severe psoriasis, who had been unresponsive to other treatments. The CysA dose ranged from 2 to 4 mg/kg/day, for periods varying from 8 to 105 weeks (mean: 54 weeks). Dose tapering was gradual after lesion improvement and adjusted according to clinical response. Adjuvant therapy with topical steroids, vitamin D3 ointments, coal tar preparations or anthralin was used in all children. Acitretin was used in three patients for short periods. The children were regularly monitored for serum renal and liver function and blood pressure. Improvement of skin lesions was achieved after between 4 and 30 (mean: 12) weeks of treatment, with complete remission in three children. Relapse of lesions occurred in the other children during CysA reduction, but they responded to a dose increase. The treatment was found to be well tolerated and with no significant side-effects. CysA can be used in carefully selected and monitored patients and may represent an alternative tool for severe episodes of psoriasis in children, when other therapies are unsuccessful.
- Ecthyma gangrenosum secondary to severe invasive infection caused by Escherichia coli.Publication . Gomes, J; Vilarinho, C; Ventura, F; Vieira, AP; Brito, C
- Edema hemorrágico agudo infantilPublication . Pereira, T; Nunes, S; Vieira, AP; Sá, C; Pereira, A; Sousa-Basto, AO edema hemorrágico agudo infantil é uma forma rara de vasculite leucocitoclásica cutânea, que ocorre em crianças com menos de 4 anos de idade. As principais manifestações são lesões cutâneas purpúricas, edema periférico e febre. Apesar dos achados clínicos serem dramáticos, quer na aparência das lesões, quer na rapidez de instalação, o prognóstico permanece excelente, com recuperação espontânea em poucas semanas. A sua origem está pouco esclarecida, mas infecções subjacentes, fármacos e vacinas têm sido referidas como possíveis factores etiológicos. Descrevemos uma criança de 7 meses com quadro clínico e histológico típicos de edema hemorrágico agudo infantil que surgiu na sequência de uma infecção do tracto urinário, em tratamento com amoxicilina e ácido clavulâmico.
- Hypotrichosis with Juvenile Macular DystrophyPublication . Almeida, FT; Carneiro-Freitas, R; Caldas, R; Vieira, APHypotrichosis with juvenile macular dystrophy is a rare autosomal recessive disease, characterized by hypotrichosis and progressive macular degeneration, leading to blindness in the first three decades of life. It is associated with mutations in the cadherin 3 gene, resulting in the abnormal expression of P-cadherin. We report a case of a 4-year-old female patient diagnosed with this genodermatosis.
- Imunoglobulinas endovenosas em dermatologia – experiência clínica de 7 anos no Hospital de BragaPublication . Araújo, C; Fernandes, JC; Duarte, ML; Pereira, T; Vieira, AP; Brito, CIntrodução: Nos últimos anos tem aumentado a experiência clínica com o uso de Imunoglobulinas Endovenosas (IgEv) em Dermatologia. Apesar da informação limitada na literatura, a utilização off-label das IgEv tem demonstrado eficácia na terapêutica de várias dermatoses refratárias aos tratamentos convencionais. Material e métodos: Efetuou-se um estudo retrospetivo dos doentes com patologia dermatológica tratados com IgEv entre Janeiro de 2004 e Outubro de 2011 no Serviço de Dermatologia do Hospital de Braga. Foram analisadas as características demográficas e clínicas, as terapêuticas efetuadas, a resposta clínica e o perfil de segurança. Resultados: Foram tratados 21 doentes com IgEv em 10 diferentes patologias dermatológicas: quatro doentes com Pênfigo Vulgar [2 com resposta completa (RC), um com resposta parcial (RP) e outro que interrompeu o tratamento por efeito lateral grave]; dois doentes com Penfigóide Bolhoso (um com RC e outro com RP); três doentes com Necrólise Epidérmica Tóxica (NET) com RC; dois doentes com Dermatomiosite (ambos com RP); quatro doentes com Urticária Crónica (um com RC, um com RP, um que não respondeu e outro que suspendeu o tratamento por efeito lateral); dois doentes com Vasculopatia Livedóide com RP; um doente com Síndrome CREST que não melhorou; um doente com Escleromixedema com RP; um doente com Pioderma Gangrenoso com RC e uma doente com Dermite Atópica que interrompeu o tratamento na sequência de gravidez. Com excepção dos 3 doentes com NET, em todos os outros a doença havia sido refratária aos tratamentos sistémicos convencionais. Conclusões: Apesar de a nossa experiência ser limitada, o tratamento com IgEv pode ser benéfico em determinadas patologias que não melhoram com o tratamento clássico. Atendendo ao seu custo elevado e efeito terapêutico variável, o seu uso deve ser criterioso até que mais estudos definam a relação risco-benefício.