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Publication
Eritrodermia: Primeira Manifestação de Défice Imunitário Congénito
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Advisor(s)
Abstract(s)
A Doença do Enxerto Contra o Hospedeiro associada a transfusão (DEH-AT) é uma complicação rara da
transfusão de hemoderivados não irradiados que afecta habitualmente indivíduos pertencentes a grupo de risco, nomeadamente crianças com imunodeficiência congénita. Caracteriza-se por um envolvimento cutâneo, gastrointestinal e hematológico, sendo habitualmente fatal.
Descreve-se um caso DEH-AT atenuada que constituiu o indício para o diagnóstico de uma imunodeficiência combinada. A doença manifestou-se com rash característico mas não apresentou envolvimento digestivo ou hematológico, provavelmente pela corticoterapia concomitante. Tal facto permitiu a sobrevivência da criança e o diagnóstico de uma imunodeficência combinada. O diagnóstico definitivo de DEH-AT pode ser difícil uma vez que as manifestações clínicas e histológicas mimetizam, com frequência, outras situações, tais como toxidermia ou doença viral.
Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunode ficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. We report a case of a nonlethal, attenuated, TA-GVHD which was the clue to the diagnosis of combined immunodeficiency. The disease was marked by the presence of a severe rash but lacked all the other usual manifestations and wasn’t fatal due to the fact that this child was under high-dose corticotherapy. This led to the survival of this child and allowed the diagnosis of a combined immunodeficiency. The definitive diagnosis of GVHD can be problematic because the clinical and histological features can mimic other conditions such as drug eruptions, viral rash or eczema.
Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunode ficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. We report a case of a nonlethal, attenuated, TA-GVHD which was the clue to the diagnosis of combined immunodeficiency. The disease was marked by the presence of a severe rash but lacked all the other usual manifestations and wasn’t fatal due to the fact that this child was under high-dose corticotherapy. This led to the survival of this child and allowed the diagnosis of a combined immunodeficiency. The definitive diagnosis of GVHD can be problematic because the clinical and histological features can mimic other conditions such as drug eruptions, viral rash or eczema.
Description
Keywords
HSAC DER HDE INF PED Doença Enxerto-Hospedeiro Reação Transfusional Eritrodermia
Citation
Trab Soc Port Dermatol Venereol 2011;69(1):97-101
Publisher
Sociedade Portuguesa de Dermatologia e Venereologia