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European Journal of Dermatology

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Congenital erythroderma should be considered as an urgent warning sign of immunodeficiency: a case of Omenn syndrome Volume 27, numéro 3, May-June 2017

Illustrations


  • Figure 1
Auteurs
1 Erasmus Medical Centre,
Department of Dermatology,
Rotterdam,
The Netherlands
2 University Medical Centre Utrecht and Wilhelmina's Children Hospital,
Department of Pediatric Immunology and Infectious Diseases,
Utrecht,
The Netherlands
3 University Medical Centre Utrecht,
Department of Medical Genetics,
Utrecht,
the Netherlands
4 Elisabeth-Tweesteden Hospital,
Department of Dermatology,
Tilburg,
The Netherlands
5 Elisabeth-Tweesteden Hospital,
Department of Pediatrics,
Tilburg,
The Netherlands
6 University Medical Centre Utrecht,
Department of Pathology,
Utrecht,
The Netherlands
7 University Medical Centre Utrecht,
Department of Dermatology,
Utrecht,
The Netherlands
8 University Medical Centre Utrecht and Wilhelmina's Children Hospital,
Department of Immunology,
Utrecht,
The Netherlands

A four-week old girl born to non-consanguineous Dutch parents after an uneventful pregnancy was referred with congenital erythroderma. Erythroderma with large scales and alopecia of the scalp, eyelashes, and eyebrows was observed (figure 1A), without lymphadenopathy or hepatosplenomegaly. One pustule on the forehead was present, without clinical evidence of infection. Failure to thrive directly after birth was attributed to increased caloric requirement, loss of protein secondary to skin changes, [...]