Wednesday, 7 December 2011
Poster Hall (Cancún Center)
Aura Pellizzeri, MD
,
Inmunology and Allergolgy, Resident, Caracas, Venezuela
Durnes García, MD
,
Inmunology and Allergolgy, Teaching Coordinator, Caracas, Venezuela
Joselit Torres, MD
,
Inmunology and Allergolgy, Resident, Caracas, Venezuela
Mayra Martínez, MD
,
Inmunology and Allergolgy, Resident, Caracas, Venezuela
Elianny Chavez, MD
,
Inmunology and Allergolgy, Resident, Caracas, Venezuela
Diana Briceño, MD
,
Inmunology and Allergolgy, Attending, Caracas, Venezuela
Background: Neonatal Lupus is a rare disease, described in 1954, which only occurs in children of mothers with antibodies to specific antigens of Systemic Lupus Erythematosus (SLE). Such antigens pass, transplacentally during gestation and is characterized by cutaneous manifestations such as rash, erythematous macules, papules or plaques which tend to coalesce; less frequent are blood disorders including aplastic anemia, neutropenia, thrombocytopenia, hemolytic anemia, pancytopenia; liver disorders with elevated liver enzymes and cholestasis; central nervous system disease such as mielopaias, convulsions; pulmonary as pneumonitis, and gastrointestinal tract such as bloody diarrhea. Alopecia is a common symptom in SLE but has not been reported in the literature in neonatal presentation.
Methods: Case report: A 1 month and 4 day old infant female , who presented 3 days after birth with a persistent bloody diarrhea , without mucus also presents dehydration and metabolic acidosis that warrant intravenous correction. An infectious etiology is discarded and is referred to an allergist for study of a possible lactose intolerance which is discarded initially. However, the physical finding of hair loss is evident with areas of alopecia which together with the persistence of the diarrhea and rash is suspect of a possible immunological etiology. Therefore, it was decided to test Anti-Ro autoimmunity in the infant and his mother; given a positive results
Results: With the Anti-Ro test the presence of Neonatal Lupus is confirmed. A treatment with EV-dose methylprednisolone was initiated, which had little clinical response, meriting a treatment with azathioprine with a good clinical response, which improved and reduced Anti-Ro values.
Conclusions: The importance of clinical observation is evident when there are unusual features which allows for a rare diagnosis. This striking case, given the unusual presentation with alopecia which together with the clinical observations was indicative of this disease, and not of other syndromes characteristic of this age, like sepsis.
