Hyper-IgE syndrome (HIES) is a primary immunodeficiency disorder characterized by eczema, cold abscesses, pneumonia, eosinophilia, and a very high serum IgE concentration. An association with celiac disease is rare. Immunodeficiency and autoimmunity are two manifestations of immune system dysfunction that can be associated with common pathophysiological links. We present the case of a 3-year-old child with psychomotor retardation and a history of recurrent infections who had generalized eczema, failure to thrive, and abdominal distension with hepatosplenomegaly. The patient improved after receiving a monthly intravenous immunoglobulin infusion and a gluten-free diet.
La pemphigoïde gestationis est une dermatose gravidique apparaissant en général entre la 28ème et la 32ème semaine d'aménorrhée. L’éruption cutanée est prurigineuse, bulleuse ou vésiculopapuleuse et de topographie péri-ombilicale. Outre la clinique, son diagnostic repose sur la biopsie cutanée avec analyse en immunofluorescence directe. Elle se caractérise par sa tendance à récidiver. Le traitement habituel nécessite des dermocorticoïdes. Les conséquences peuvent être maternelles (menace d'accouchement prématuré), fœtales (retard de croissance intra-utérin), et néo-natales (éruption cutanée). Nous rapportons un cas de pemphigoïde gestationis associé à une aplasie cutanée congénitale type 2. S'agit il d'une association fortuite?
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