Epidermolysis bullosa (EB) is a rare inherited disease characterized by vesiculobullous lesion that arise in response to minimal trauma or shearing force to the skin. Epiderrnolysis bullosa herpetiformis-Dowling Meara, a variant of EB, has its onset at birth and can be a life threatening disease as it was in this case. We report a case of epidermolysis bullosa herpetiformis in a newborn with a generalized intraepidermal blisters noted at birth whose diagnosis was confirmed by electron microscope. A brief review of the literature is included.
Blister ; Diagnosis ; Epidermolysis Bullosa* ; Humans ; Infant, Newborn ; Parturition ; Skin

PURPOSE: To determine clinical and hematological characteristics of infants born to pregnancy-induced hypertensive mothers and whether neutropenia in these infants is associated with an increased incidence of neonatal sepsis. METHODS: A retrospective study was conducted from June 1995 to June 1997 in 84 infants of pregnancy-induced hypertensive mothers who were admitted to the neonatal intensive care unit of Asan Medical Center. These infants were divided into 2 groups according to their absolute neutrophil counts(Group I: with neutropenia, Group II: without neutropenia) and their clinical, hematological and maternal characteristics were compared between these groups. RESULTS: 1) Infants in Group I were smaller, younger, and delivered more by cesarean section than in infants in Group II. 2) Neutropenia was observed in 77,7% of infants who were less than 30 weeks of gestational age and less than 1,500 g. Neutropenia and thrombocytopenia seem to be a transient phenomenon improving spontaneously approximately after 5.8 days and 8-10 days, respectively. 3) Sex, 1 min apgar score, type of delivery and initial use of antibiotics differ between these 2 groups. 4) There was no apparent increased risk for development of neonatal sepsis associated with neutropenia. CONCLUSION: Neutropenia and thrombocytopenia were observed in 40-50% infants born to pregnancy-induced hypertensive mothers. Such finding was more pronounced in infants whose gestational age was less than 32 weeks and birth weight was less than 1,500 g occuring at 70-80%. Neutropenia, per se, is not associated with increased incidence of sepsis but changes in hematological findings and clinical evidence is more important in predicting sepsis in these infants.
Anti-Bacterial Agents ; Apgar Score ; Birth Weight ; Cesarean Section ; Chungcheongnam-do ; Female ; Gestational Age ; Humans ; Hypertension, Pregnancy-Induced ; Incidence ; Infant ; Infant, Newborn* ; Intensive Care, Neonatal ; Mothers* ; Neutropenia ; Neutrophils ; Pregnancy ; Retrospective Studies ; Sepsis ; Thrombocytopenia ; Thrombocytopenia, Neonatal Alloimmune