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Gabriela Torres and Carla Rosero
Central University of Ecuador, Ecuador
ScientificTracks Abstracts: J Skin
This case report describes about a syndrome of recurrent staphylococcal abscesses, sinopulmonary infections, and severe eczema. The condition was initially called Job syndrome. It was speculated that the immune defect in Job syndrome lay in an abnormality in nonspecific mechanisms of local bacterial resistance and perhaps in abnormalities of mediators of the acute inflammatory response. This disorder was termed hyperimmunoglobulin E syndrome (HIES) when an associated increase in serum levels of immunoglobulin (Ig) E was described. HIES is rare; the precise incidence is not known. It is found equally among males and females, and is observed in members of succeeding generations. It has been reported in Caucasians as well as in individuals of Asian and African origin. HIES is characterized by dermatitis, recurrent infections (principally bacterial), and elevated serum IgE, although there is significant variation in the constellation of symptoms and signs among individual patients. Classification of HIES into two subtypes has been proposed. We present an 11-year-old male patient with a history of recurrent pneumonia, a history of cutaneous eczema that begins at birth, with severe to mild seizures; presents prominent front, broad nasal bridge, hypertelorism, fleshy nose, prognathism, statural decrease and scoliosis. It was detected: IgE of 2620. The clinical scale of Grimbacher criteria was applied to the patient, scoring 47 points with a high probability of diagnosis of Hyper Ig Syndrome. It was treated with short and sporadic cycles of steroids, antihistamines, vitamin C and antibiotic therapy, 1 monthly dose. He received three sessions of phototherapy weekly, achieving clinical improvement of the dermatological picture for several months; nevertheless the patient dies from complications due to a new episode of pneumonia.
Gabby Torres has completed his PhD in Medicine and Surgery from the Central University of Ecuador. She has taken her specialization in Dermatology from University of Guayaquil. She is the Member of the Ecuadorian Society of Dermatology. She has named as the Best Ecuadorian Dermatologist by the International Organization for Training and Medical Research. she has received the first place in the second contest for Ecuadorian Dermatological Research. She is the University Assistant of the Coordination of the Postgraduate of Dermatology at the Central University of Ecuador. She is working as a Physician and the Head of Teaching at Hospital Gonzalo Gonzalez. She is the Member of the Ibero-Latin American College of Dermatology, Bolivarian Society of Dermatology and Mesoamerican Academy of Dermatology and Cosmetic Surgery.
