Autoimmune Thyroiditis Presenting as Palmoplantar Keratoderma

Palmoplantar keratoderma is a heterogeneous group of hereditary and acquired disorders characterized by abnormal thickening of palms and soles. Hypothyroidism is an unusual cause of palmoplantar keratoderma, rarely reported in the literature. We report a case of a 43-year-old woman presented with a 3-month history of a diffuse palmoplantar hyperkeratosis unresponsive to topical keratolytics and corticosteroids. Her past medical and family histories were unremarkable. She complained of recent asthenia, mood changes and constipation. Laboratory evaluation revealed an autoimmune thyroiditis with hypothyroidism. Other causes of acquired palmoplantar keratoderma were excluded. After hormonal replacement therapy institution, a gradual improvement of skin condition was observed. The diagnosis of underlying causes for acquired palmoplantar keratoderma can be a difficult task; however its recognition is essential for successful treatment results. Although a very rare association, hypothyroidism must be suspected in patients with acquired palmoplantar keratoderma, particularly when it occurs in association with systemic symptoms

Angiohistiocitoma de Células Multinucleadas

Multinucleated cell angiohistiocytoma (MCAH) is a rare clinical entity whose pathogenesis is not fully understood. It is more common in middle-aged women and is characterized by erythematoviolaceous papules or nodules, usually asymptomatic and predominantly located in the extremities. Histopathology is essential for making the diagnosis, consisting of typical alterations in the dermis, namely the proliferation of small vessels and the presence of multinucleated giant cells. Evolution is benign, although persistent, with few reported cases of spontaneous remission. We present the case of a 48-year-old man with MCAH, who exhibited asymptomatic papules and nodules grouped on the back of both hands, with a year of evolution. The diagnosis was made after histopathology, which excluded other differential diagnoses. With this case report, we intend to contribute to the knowledge about this entity and highlight the interconnection of clinical observation and histopathologic analysis.O angiohistiocitoma de células multinucleadas (ACM) é uma entidade rara, cuja patogenia não está completamente compreendida. É mais comum em mulheres de meia-idade e caracteriza-se por pápulas ou nódulos eritematovioláceos, habitualmente assintomáticos e localizados predominantemente nas extremidades. O diagnóstico é fundamentalmente histopatológico, consistindo em alterações típicas na derme, nomeadamente na proliferação de pequenos vasos e na presença de células gigantes multinucleadas. A sua evolução é benigna, embora persistente, havendo poucos casos relatados de remissão espontânea. Apresenta-se o caso de um homem de 48 anos com diagnóstico de ACM, manifestando-se por pápulas e nódulos assintomáticos, agrupados no dorso de ambas as mãos, com um ano de evolução. O diagnóstico foi determinado pelo exame histopatológico, excluindo-se outros diagnósticos diferenciais. Com este relato de caso pretende-se contribuir para o conhecimento sobre esta patologia e destacar a complementaridade entre a observação clínica e a análise histopatológica

TINHAS DO COURO CABELUDO – ESTUDO RETROSPETIVO DE 5 ANOS (2008-2012) NO HOSPITAL SANTO ANTÓNIO DOS CAPUCHOS

Introduction: Tinea capitis is the most common fungal infection in children. Recent studies have shown important epidemiological differences in Portugal, particularly with respect to the dermatophytes responsible for tinea capitis.Methods: Our study aimed to characterize the epidemiology of tinea capitis diagnosed at the Dermatology department of Hospital dos Capuchos between 2008 and 2012, through a retrospective analysis of the results of cultures performed in patients with suspected tinea capitis during this period.Results: We studied 935 samples with 556 (59.3%) positive cultures. The study population had an average age of 5.4 years and was predominantly male (63.7%) and black (81.3%). We isolated eight species of dermatophytes, and M. audouinii was the dominant specie (57.7%), followed by T. soudanense (28.8%). Infections by M. audouinii and T. soudanense occurred mostly in black patients (80.1% and 53.8%, respectively). In turn, infection by M. canis was more common in caucasians (76.9%).Conclusion: This study showed a clear predominance of anthropophilic species, with M. audouinii and T.soudanense being responsible for about 87% of tinea capitis. The fact that our patients are mostly of the black race may explain these results, as well as the differences when compared our study to other studies carried out in Portugal.Introdução: A tinha do couro cabeludo é a infeção fúngica mais frequente em crianças. Estudos recentes, realizados em Portugal, têm demonstrado diferenças epidemiológicas regionais importantes, em particular no que diz respeito aos dermatófitos responsáveis pelas tinhas do couro cabeludo.Métodos: O nosso estudo teve como objetivo a caracterização epidemiológica das tinhas do couro cabeludo diagnosticadas no Serviço de Dermatologia do Hospital dos Capuchos entre 2008 e 2012 através da análise retrospetiva dos resultados das culturas realizadas em doentes com suspeita de tinha do couro cabeludo durante este período.Resultados: Foram estudadas 935 amostras, com 556 (59,3%) culturas positivas. A população estudada tinha uma idade média de 5,4 anos e era predominantemente do sexo masculino (63,7%) e de raça negra (81,3%). Foram isoladas oito espécies de dermatófitos, sendo que, M. audouinii foi a espécie dominante (57,7%), seguido de T. soudanense (28,8%). Tanto as infeções por M. audouinii como por T. soudanense ocorreram maioritariamente em doentes de raça negra (80,1% e 53,8%, respetivamente). Por sua vez, a infeção por M. canis, foi mais comum na raça caucasiana (76,9%).Conclusão: Este estudo demonstrou um claro predomínio de espécies antropofílicas, sendo o M. audouinii e T. soudanense responsáveis por cerca de 87% das tinhas do couro cabeludo. O facto de a nossa população ser, maioritariamente, de raça negra pode justificar estes resultados, bem como, as diferenças encontradas em relação a outros estudos realizados em Portugal

Purpura: primary systemic amyloidosis manifestation

A Amiloidose Sistémica Primária (AL) é a forma mais frequente de amiloidose sistémica e a sua morbilidade está associada à deposição de cadeias leves de imunoglobulinas em órgãos vitais. As manifestações mucocutâneas ocorrem em cerca de 30-40% dos casos e são importantes na suspeição diagnóstica, uma vez que surgem precocemente na evolução da doença. Descreve-se caso de uma doente do sexo feminino, 71 anos, com lesões purpúricas disseminadas e fragilidade cutânea com seis meses de evolução, acompanhada por queixas de disfagia. A avaliação laboratorial inicial não mostrava alterações. O estudo histológico e imunohistoquímico da amostra da gordura abdominal e da biópsia cutânea revelaram a presença de substância amilóide com marcação para cadeias leves lambda. Da avaliação efectuada, salienta-se proteinúria > 1g/24h com proteínas de Bence-Jones e a presença de banda monoclonal de cadeias leves lambda na imunoelectroforese sérica. Com o diagnóstico de amiloidose AL, foi iniciado tratamento com melfalan e prednisolona. Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.publishersversionpublishe

UTILIZAÇÃO OFF-LABEL DE TERAPÊUTICA BIOLÓGICA EM DERMATOLOGIA – EXPERIÊNCIA CLÍNICA DE 5 ANOS

Introduction: In recent years, the development of numerous biologic agents extended the therapeutic armamentarium available to dermatologists. Although they are currently approved only for psoriasis, experience is being accumulated with its use in the treatment of different inflammatory skin diseases.Methods: This was a retrospective study of all patients treated in our department with biologic agents for skin disease other than psoriasis. We analyzed the clinical data, previous and concomitant treatments, efficacy and safety profile of these agents in this setting.Results: 15 patients were included with 7 different skin diseases resistant to conventional therapies. Etanercept was used in 4 cases: 3 patients with scleroderma (2 patients developed major side effects and had to interrupt treatment, while the third patient had a good response) and one patient with elastolytic granuloma. Four patients were treated successfully with infliximab (3 cases of Behçet’s disease and 1 case of pytiriasis rubra pilaris). Adalimumab was used to treat a patient with subcorneal pustular dermatosis with excellent results. Efalizumab was ineffective in 2 cases of atopic dermatitis. Rituximab was used in 5 patients: 3 cases of pemphigus (with complete clinical improvement) and 2 patients with atopic dermatitis (1 patient had a good response but had to interrupt treatment because she become pregnant and in the other patient there wasn’t any clinical improvement).Conclusion: Biologic immunomodulators have demonstrated efficacy in the treatment of several dermatologic diseases; however, most of the available information are individual case reports or small case series. Despite our limited experience in this setting, these results seem promising in the treatment of some inflammatory skin diseases resistant to conventional therapies.Introdução: Nos últimos anos, a terapêutica biológica expandiu de forma expressiva as opções terapêuticas disponíveis em Dermatologia. Ainda que o seu uso esteja apenas aprovado no tratamento da psoríase e psoríase atropática, a utilização off-label em diversas dermatoses inflamatórias tem sido descrita de forma crescente na literatura.Métodos: Efectuou-se um estudo retrospectivo dos doentes com dermatoses (que não a psoríase) tratados com terapêutica biológica entre Janeiro de 2005 e Dezembro de 2009, no nosso Serviço. Foram analisados os dados clínicos, as terapêuticas efectuadas, a eficácia e o perfil de segurança.Resultados: Foram incluídos 15 doentes e tratadas 7 diferentes patologias dermatológicas resistentes às terapêuticas convencionais. O etanercept foi utilizado em 4 casos: 3 doentes com Esclerodermia sistémica (2 doentes interromperam a terapêutica antes das 12 semanas por efeitos adversos graves; no 3º doente verificaram-se bons resultados) e um doente com granuloma elastolítico, no qual se registou uma boa resposta clínica. O infliximab foi utilizado com sucesso em 4 doentes (3 casos de doença de Behçet e 1 de pitiríase rubra pilar). O adalimumab foi utilizado num caso de dermatose pustulosa subcórnea com excelentes resultados. O efalizumab foi ineficaz em 2 casos de dermite atópica. O rituximab foi utilizado em 5 doentes: 3 casos de pênfigo (com excelentes resultados terapêuticos) e 2 casos de dermite atópica (numa doente houve uma boa resposta, mas interrompeu a terapêutica após 1 semana de tratamento por gravidez e na 2ª doente não se observou qualquer melhoria significativa).Conclusão: A terapêutica biológica tem demonstrado eficácia no tratamento de várias dermatoses; no entanto a maioria da informação disponível na literatura é referente a casos isolados ou a pequenas séries de casos. Apesar da nossa experiência ser limitada, estes resultados parecem-nos promissores na terapêutica de determinadas dermatoses inflamatórias refractárias aos tratamentos convencionais

Conscious sedation with inhaled 50% nitrous oxide/oxygen premix in photodynamic therapy sessions for vulvar lichen sclerosus treatment

Photodynamic therapy has been described as an effective therapeutic option in selected cases of anogenital lichen sclerosus that are refractory to first-line treatments. However, procedure-related pain is a limiting factor in patient adherence to treatment. The authors report the case of a 75-year-old woman with highly symptomatic vulvar lichen sclerosus, successfully treated with photodynamic therapy. An inhaled 50% nitrous oxide/oxygen premix was administered during sessions, producing a pain-relieving, anxiolytic, and sedative effect without loss of consciousness. This ready-to-use gas mixture may be a well-tolerated and accepted alternative to classical anesthetics in Photodynamic therapy, facilitating patients' adherence to illumination of pain-prone areas

Onychomycosis in patients with chronic leg ulcer and toenail abnormalities

Nails have a limited number of reactive patterns to disease. Accordingly, toenail changes of different etiologies may mimic onychomycosis. OBJECTIVE To determine the prevalence of toenail onychomycosis among patients with leg ulcer and toenail abnormalities attending a dermatology clinic. METHODS A cross-sectional study was conducted through the analysis of clinical records and results of mycological examination. RESULTS A total of 81 patients were included, with a median age of 76.0 years. Most ulcers were of venous etiology, followed by those of mixed and arterial pathogenesis. The mycological evaluation confirmed the diagnosis of onychomycosis in 27.2% of the patients. The etiologic agent was a dermatophyte in 59.1% of isolates in nail samples, while Trichophyton interdigitale was the most frequent fungal species (40.9%). CONCLUSIONS Most toenail abnormalities in patients with chronic leg ulcer were not onychomycosis. This study highlights the importance of systematic mycological examination in these patients, in order to avoid overtreatment with systemic antifungals, unnecessary costs and side effects