Journal of the Portuguese Society of Dermatology and Venereology
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A tricoscopia como Pista para o Diagnóstico
Get PDFA 43-year-old man presented to our department with generalized erythroderma since early childhood, previously treated with topical and systemic corticosteroids with only partial improvement. The physical examination revealed polycyclic erythematous scaly plaques on the trunk and extremities and lichenification of the body folds (Fig. 1). In addition to skin lesions, we observed sparse hair of both eyebrows and eyelashes, however hair of the scalp was normal. The patient had a history of asthma in childhood. The family history was non- -contributory and the patient didn’t have any children. Analytically, routine blood tests were normal, however an elevated serum level of IgE 29650 UI/mL (N < 165) was detected. We performed trichoscopy of the eyebrows that showed nodules along the hair shaft and distal fractures (Fig.s 2a and 2b). The trichoscopic examination of the hair of the scalp did not identify any changes.
Dermatose Degenerativa Induzida por D-penicilamina em Paciente com Doença de Wilson
Get PDFD-penicillamine induced degenerative dermatosis include, among others, elastosis perforans serpiginosa, and pseudo- -pseudoxanthoma elasticum. Elastosis perforans serpiginosa is a rare perforating disease characterized by transepidermal elimination of abnormal elastic fibers. This condition can be idiopathic, reactive or induced by D-penicillamine, commonly used for the treatment of Wilson disease, cystinuria, rheumatoid arthritis or systemic sclerosis. Cutaneous manifestations resembling pseudoxanthoma elasticum but lacking familiar history and ABCC6 mutations have been identified as a D-penicillamine induced dermopathy and called pseudo-pseudoxanthoma elasticum. The authors present a 17-year-old caucasian female treated for several years with D-penicillamine for Wilson disease who developed asymptomatic papules, some hyperkeratotic skin-colored and other soft and yellowish, on the cervical region and face. Histopathology showed transepidermal elimination of numerous, branched, sawtooth-like elastic fibers. These findings suggested a D-penicillamine induced dermopathy and the authors considered the diagnosis of both elastosis perforans serpiginosa and pseudo- -pseudoxanthoma elasticum in the same patient. The drug was switched to zinc acetate. No newer lesions appeared thereafter but previous lesions persisted at the 1 year follow-up.As dermatoses degenerativas induzidas por D-penicilamina incluem, entre outras, a elastose perfurante serpiginosa e o pseudo-pseudoxantoma elástico. A elastose perfurante serpiginosa é uma doença perfurante rara caracterizada pela eliminação transepidérmica de fibras elásticas anormais. Esta condição pode ser idiopática, reativa ou induzida por D-penicilamina, habitualmente utilizada para o tratamento da doença de Wilson, cistinúria, artrite reumatóide ou esclerose sistémica. Manifestações cutâneas semelhantes a pseudoxantoma elástico mas sem história familiar e mutações do gene ABCC6 foram identificadas como sendo uma dermatose induzida por D-penicilamina e designada de pseudo-pseudoxantoma elástico. Descreve-se o caso de uma mulher de 17 anos tratada por vários anos com D-penicilamina para doença de Wilson, com pápulas assintomáticas, algumas cor de pele e hiperqueratósicas e outras macias e amareladas, na região cervical e face. A histopatologia mostrou a eliminação transepidérmica de fibras elásticas espessadas, em forma de dentes de serra. Estes achados sugeriram uma dermopatia induzida por D-penicilamina e os autores consideraram o diagnóstico de elastose perfurante serpiginosa e pseudo-pseudoxantoma elástico no mesmo paciente. O fármaco foi alterado para acetato de zinco sem lesões novas, mas com manutenção das lesões existentes no seguimento a 1 ano
Reconstrução da Região Labial Inferior: Um Exemplo de Versatilidade do Retalho Nasogeniano
Get PDFThe nasolabial flap represents a valid and well known option to reconstruct defects of the lower lip as in the following case. An 82-year-old male patient with a basal cell carcinoma located on the right lower lip skin had a surgical excision under local anesthesia. The large surgical defect was closed with an inferiorly based pedicle nasolabial flap, with no flap necrosis and acceptable functional and aesthetic results.O retalho nasogeniano de pedículo inferior representa uma opção válida e bem reconhecida para reconstrução de defeitos do lábio inferior, tal como no caso que descrevemos. Homem, 82 anos, com carcinoma basocelular do 1/3 direito da pele do lábio inferior, sem envolvimento da mucosa. Sob anestesia local, a lesão foi excisada com margem cirúrgica adequada, tendo-se gerado um defeito elipsoide complexo. Procedeu-se ao encerramento do mesmo com retalho nasogeniano de pedículo inferior. Não houve necrose no pós-operatório tardio. Os resultados estéticos e funcionais foram aceitáveis
Um caso de Queratodermia Aquagénica Plantar
Get PDFAquagenic keratodermais a rare acquired disorder, most prevalent in young female patients, affecting mainly palmar and plantar regions. Clinically, it presents as translucent edematous papules that after a brief immersion in water turn into a wrinkling skin surface, and resolve after drying the affected region. There are associations with several diseases and Cystic Fibrosis is the most related. We report a peculiar clinical case of the disease, with unilateral edematous hypochromic plaque on left heel with good response to treatment with 3% salycilic acid in vaseline. There was no respiratory involvement.A queratodermia aquagénica é uma doença adquirida rara, predominante em pacientes jovens do sexo feminino, acometendo, principalmente, palmas e, mais raramente, as plantas dos pés. Clinicamente, apresenta-se com pápulas translúcidas, edematosas, tornando a pele com aspecto enrugado, após breve contato com água e a sua resolução ocorre após a secagem do local. Há associações com várias doenças, sendo a fibrose cística a mais destacada. Apresentamos um caso clínico peculiar da doença, com placa hipocrómica edematosa unilateral no calcanhar esquerdo, com boa resposta ao tratamento com vaselina salicilada a 3%
Dermatoscopia do Carcinoma de Células de Merkel- Relato de 1 caso
No full textMerkel cell carcinoma is a rare neuroendocrine tumor localized in photo-exposed areas in aged adults. A high risk of local recurrence, early metastatic disease and, frequently, a late diagnosis, are determinant of a poor prognosis. Dermoscopic examination of this lesion, which is characterized by the presence of multiple atypical vessels and “red-milky areas”, might be a very useful tool to suspect the diagnosis rapidly and early, allowing treatment in due time. The authors report the case of a 71-years-old patient presenting with a Merkel cell carcinoma in the right lower limb in which the dermoscopic findings picked up a high grade of suspicion and allowed tumor excision in a clinically useful and treatable time. The authors also describe the most important dermoscopic findings and patternsobserved in this tumor and the differential diagnosis one must consider in these cases.O carcinoma de células de Merkel é um tumor neuro-endócrino pouco frequente localizado principalmente nas áreas foto-expostas de adultos de idade avançada. Fatores como o elevado risco de recidiva local e doença metastática somados a um diagnóstico frequentemente tardio, são determinantes de um mau prognóstico. O exame dermatoscópico desta lesão que se caracteriza pela presença de múltiplos vasos atípicos e “áreas vermelho-leitosas”, pode ser uma ferramenta útil para o estabelecimento precoce e rápido de uma suspeita diagnóstica permitindo o tratamento atempado. Os autores reportam um caso de uma doente de 71 anos com um carcinoma de células de Merkel no membro inferior direito em que os achados dermatoscópicos identificaram um elevado índice de suspeição permitindo a excisão do tumor em tempo útil. Descrevem-se os principais padrões dermatoscópicos observados e os diagnósticos diferenciais a considerar
Tratamento da Dermite Atópica Moderada a Grave em Adultos
Get PDFAtopic dermatitis is a chronic inflammatory skin condition characterized by relapsing pruritic, scaly, erythematous papules and plaques frequently associated with superinfection. It is one of the most common skin diseases affecting 10%-20% of children and 1%-3% of adults, and it is a significant cause of morbidity, quality-of-life impairment and health-care costs. Although many patients can be treated satisfactorily with topical medications and phototherapy, a smaller subset requires more aggressive systemic therapies to control skin inflammation, reduce symptoms, prevent flares, and improve quality of life. Besides, severe atopic dermatitis has a profound effect on many aspects of the patient's life, and a combination of topical and systemic treatment is often required to control the disease, after ensuring all treatment failure causes are adequately excluded. Despite the available classical options, effective and safe treatments for patients with moderate-to-severe atopic dermatitis are limited due to toxicity and side effects. This article reviews the use and the scientific evidence of these medications in the treatment of adult moderate to severe atopic dermatitis, as well as several promising targeted therapies currently in development.A dermite atópica é uma doença cutânea inflamatória crónica, caracterizada por lesões eritemato-descamativas pruriginosas recorrentes, frequentemente associadas a sobreinfecção. É uma das mais comuns doenças cutâneas, afectando 10% a 20% das crianças e 1% a 3% dos adultos, sendo uma causa significativa de morbilidade, compromisso de qualidade de vida e consumo de cuidados de saúde. Ainda que muitos doentes possam ser tratados de forma satisfatória com medicação tópica e fototerapia, um subgrupo de doentes requer terapêutica sistémica mais agressiva para controlar a inflamação cutânea, reduzir sintomas, prevenir exacerbações e melhorar a qualidade de vida. Para além disso, as formas graves da doença têm um profundo impacto na vida dos doentes o que requer a combinação de tratamentos tópicos e sistémicos para as controlar, depois de adequadamente excluídos os motivos para falência terapêutica. Apesar das múltiplas opções clássicas disponíveis, terapêuticas eficazes e seguras estão limitadas pela toxicidade cumulativa e efeitos adversos associados. Este artigo revê o uso e a evidência científica disponível destas opções de tratamento na dermite atópica moderada a grave do adulto, bem como vários novos e promissores fármacos presentemente em investigação
A Importância de Clínicas Dermatológicas Dedicadas no Atendimento de Receptores de Transplantes de Órgãos
Get PDFOrgan transplant recipients have a high risk of skin cancer associated with immunosuppressive therapy and ultraviolet radiation. The incidence of non-melanoma skin cancer, in particular is up to 65-times higher than for the normal population. Field cancerization of sun- exposed skin is also a major health issue in these patients1.In Portugal there were 895 solid organ transplants in 2017, with the majority being kidney (529) and liver (259)2. There are already several publications concerning skin cancer in Portuguese organ transplant recipients, reporting a prevalence of non-melanoma skin cancer ranging from 15% to 25% in renal transplant recipients3,4,5and 8% in one series6that included more liver transplant recipients with relatively less immunosuppression.The sunny geographical location and sun exposure habits, namely the poor knowledge and adherence to photoprotective measures, such as hats, long-sleeved clothes or sunscreen while on outdoor work or leisure activities, pose serious difficulties for skin cancer prevention in this population. In one study concerning knowledge of sun protective measures in a population of Portuguese transplant recipients7, 29% did not know that their risk of skin cancer was increased, and 25% of those who went to the beach stayed there between 11.30 and 16:00 pm. Not surprisingly, only 8% consulted a dermatologist in the first year after transplant7.Different organizations like the Skin Care in Organ Transplant Recipients - Europe (SCOPE) or the International Transplant Skin Cancer Collaborative (ITSCC) together with institutions such as the British National Institute for Health and Clinical Excellence (NICE)8,9,10, recommend initial assessment of these patients by a dermatologist and providing them with education on photoprotection and self-examination of the skin. These patients also need regular follow-up with time intervals defined by their previous history of skin cancer and the presence of field cancerization of their sun-exposed skin. In such patients with previous skin cancer and detectable field cancerization, some authors11propose three months as time interval between appointments. Dedicated or specialist dermatology clinics for organ transplantation are recommended11,12,and have also been shown to improve compliance with photoprotection13. Their introduction in the main Portuguese referral hospitals for transplantation would allow earlier dermatological care, inclusion of dermatology among the specialties that collaborate in the transplant teams and reduction of the burden of skin cancer in these patients, saving lives and costs.
Neurotequeoma Celular em Criança: Descrição de um Caso Invulgar e Breve Revisão da Literatura
Get PDFNeurothekeoma is a rare benign neoplasm of uncertain histogenesis. Progresses in immunohistochemistry have brought new insights into its cellular differentiation and origin, recognizing the possibility of a fibrohystiocitic lineage. Also, it has been subclassified histopathologically as either myxoid, cellular, or mixed type, depending on the amount of myxoid matrix and on immunohistochemical analysis. Few cases of cellular neurothekeoma have been reported. Most of them are found on the head, neck and upper extremities and mostly in young female adults. On review of literature we have not found reports regarding dermoscopic features of neurothekeoma. Herein we report an uncommon case of cellular neurothekeoma on the axilla of a 7–year-old girl, with description of its dermoscopic findings.O neurotequeoma é uma neoplasia benigna, rara, cuja histiogénese permanece incerta. Os avanços nos estudos com a imunohistoquímica, no entanto, permitiriam o reconhecimento de uma possível origem na linhagem fibrohistiocitária. Histopatologicamente são reconhecidas três variantes de acordo com a quantidade de matriz mixóide e com a análise imunohistoquímica: mixóide, celular ou misto. Os raros casos reportados, localizaram-se sobretudo na cabeça, pescoço e membros superiores, em mulheres jovens. Na revisão da literatura não há referência às características dermatoscópicas do neurotequeoma. Neste contexto, descrevemos o caso invulgar de um neurotequeoma celular, localizado na axila de uma criança de 7 anos, do sexo feminino, e respectivos achados dermatoscópicos
Disfunção Tiroideia: Uma Nova Associação com a Psoríase?
Get PDFPsoriasis is a chronic, common skin disease, which affects the patient’s quality of life to the highest degree. Several exogenous and endogenous factors may act as triggers for psoriasis, as certain hormonal changes. Previous studies have shown that thyroid hormones may be implicated in the physiopathology of psoriasis. This paper reviews the role of thyroid hormones in psoriasis as well as the current scientific evidence of a possible association between psoriasis and thyroid dysfunction.A psoríase é uma dermatose inflamatória crónica frequente com elevado impacto na qualidade de vida dos doentes. Diversos fatores exógenos e endógenos têm sido implicados na sua etiopatogénese, nomeadamente determinados distúrbios endócrinos. Estudos prévios têm demonstrado que as hormonas tiroideias podem estar implicadas na fisiopatologia da psoríase. Neste artigo será feita uma revisão do papel das hormonas tiroideias na psoríase assim como da evidência científica atual sobre uma possível associação entre psoríase e disfunção tiroideia