Abstract Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The nails become thickened and dystrophic, which is an essential characteristic of the syndrome. The diagnosis is made based on clinical findings. This study reports a case of a patient who began with changes in hair, nails and palmoplantar keratoderma in early childhood.

CONTEXTO: A trombose venosa profunda é uma doença comum em indivíduos imobilizados. A imobilização, inerente aos pacientes paraplégicos, gera a estase venosa, que é um dos fatores descritos da tríade de Virchow para o seu desenvolvimento. O trauma é a principal causa de paraplegia e, atualmente, vem aumentando a uma taxa de 4% ao ano. OBJETIVO: Avaliar a prevalência de trombose venosa profunda em paraplégicos, em que o trauma foi o agente causal da paraplegia, utilizando como método diagnóstico o eco Doppler colorido. MÉTODOS: Estudo observacional transversal, composto por 30 pacientes paraplégicos por trauma, selecionados segundo uma análise de prontuários do Serviço de Neurocirurgia de um Hospital Universitário de Curitiba e por busca ativa em associações de assistência aos deficientes físicos. A análise da prevalência de trombose venosa profunda foi efetuada pelo intervalo de confiança de 95%. RESULTADOS: O traumatismo raquimedular foi a causa da paraplegia em 29 pacientes. A causa mais frequente do trauma foi o ferimento por arma de fogo, relatado por 17 pacientes. A presença de trombose venosa profunda diagnosticada por eco Doppler colorido foi observada em 14 pacientes da amostra. Dentre estes, a veia mais acometida foi a tibial posterior, em 11 pacientes. O membro inferior esquerdo foi três vezes mais acometido que o direito. O edema foi observado em 25 indivíduos, a cianose em 14, a úlcera em oito e o aumento de temperatura local em 13. CONCLUSÃO: A trombose venosa profunda foi prevalente, ocorrendo em 46,7% dos pacientes.

BACKGROUND: Deep vein thrombosis is a common disease among people who are immobilized. Immobility is inherent to paraplegia and leads to venous stasis, which is one of the factors covered by Virchow's triad describing its development. Trauma is the primary cause of paraplegia and is currently increasing at a rate of 4% per year. OBJECTIVE: To determine the prevalence of deep vein thrombosis in paraplegic patients whose paraplegia was caused by traumas, using color Doppler ultrasonography for diagnosis. METHODS: This was a cross-sectional observational study of 30 trauma-induced paraplegia patients, selected after analysis of medical records at the neurosurgery department of a University Hospital in Curitiba, Brazil, and by a proactive survey of associations that care for the physically disabled. The prevalence of deep vein thrombosis was analyzed using 95% confidence intervals. RESULTS: Spinal cord trauma was the cause of paraplegia in 29 patients. The most common cause of trauma was gunshot wounding, reported by 17 patients. Deep vein thrombosis was diagnosed by color Doppler ultrasonography in 14 patients in the sample. The most often affected vein was the posterior tibial, in 11 patients. The left lower limb was involved three times more often than the right. Edema was observed in 25 individuals, cyanosis in 14, ulcers in 8 and localized increase in temperature in 13. CONCLUSIONS: Deep vein thrombosis was prevalent, occurring in 46.7% of the patients.