[Neonatal erythrodermia - early manifestation of Omenn's syndrome].
Ommen's syndrome is a rare, autosomal recessive disease that is characterized by symptoms of a severe combined immune deficiency, severe infections, erythrodermia, hypereosinophilia, hepatosplenomegaly, lymphadenopathy, protracted diarrhoea, hypogammaglobulinemia and elevated serum IgE. The presented case demonstrates diagnostic problems in recognizing Omenn's syndrome. These resulted predominantly from the incomplete clinical picture of the disease in its early phase. Persistent erythrodermia was the basic manifestation of the syndrome in the initial phase. Since the occurrence of the disease is extremely rare and particular symptoms are not pathognomonic, it is their co-occurrence in association with typical immunological disorders that enabled to establish the diagnosis. Through the presentation of the above case history we should like to point to the necessity of considering Omenn's syndrome in differentiating causes of erythrodermia in neonates and infants.
III Klinika Pediatrii, Instytut Centrum Zdrowia Matki Polki, Rzgowska 281/289, 93-338 Lodz, Poland. IIIKlinikaPed@pharmanet.com.pl
Teresa
T
Wozniakowska-Gesicka
Malgorzata
M
Wisniewska-Ligier
Bogdana
B
Borowska-Rybus
D003873:Dermatitis, Exfoliative / Q000209:etiology*; D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000175:diagnosis*