Long-term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children.

BACKGROUND: Autoimmune bullous dermatoses (AIBDs) in children are uncommon, and their long-term evolution remains unknown. OBJECTIVE: The aim of this retrospective study was to characterize the long-term prognosis of AIBDs that started during childhood. METHODS: We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBDs. The long-term outcome was obtained through a phone call questionnaire. RESULTS: Sixty-three patients were included from January 1993 to December 2015, 34 female and 29 males: 27 Linear immunoglobulin A disease (LAD), 12 bullous pemphigoid (BP), 12 pemphigus, 8 herpetiform dermatitis (DH) and 4 epidermolysis bullosa aquisita (EBA). The mean age was 4.7 years old. Twenty-five patients were lost during the follow-up. For the 38 remaining patients, the mean follow-up duration for all pathologies was 6.6 years. Twenty-nine of them had at least one relapse. Late relapses were observed in two cases of DH and six cases of pemphigus (7-34 months). The mean treatment duration was 30.6 months with variability according to the AIBDs. Topical corticosteroids were used alone, effectively, for seven patients and in association with other treatment in 19 patients in complete remission. Complete remission was noted in 34/38 children with a follow-up of 4.4 years (0.08-19.5). The mean duration to complete remission was 30.5 months (6-114 months). Late nasal synechiae were reported in one EBA only. There was no significant associated comorbidity, but an association with a primary immune deficiency (PID) was observed in two cases. CONCLUSION: Childhood AIBDs appear to be of good overall prognosis but a long-term follow-up is mandatory, as relapses can be late, except for BP. The use of topical corticosteroids is frequently effective alone or in association. The association with PID leads to think about the possibility of a possible underlying dysimmunity in the child.

[Subacute cutaneous lupus erythematosus and cancer: two cases and literature review]. / Lupus cutané subaigu et cancer: deux cas et revue de la littérature.

BACKGROUND: Subacute cutaneous lupus erythematosus is an uncommon dermatosis characterised by a non-scarring, annular photo-distributed dermatosis associated with anti-Ro/SSA antibodies. It is remarkable as a paraneoplastic syndrome (12 cases in the literature). We report two cases of subacute cutaneous lupus erythematosus occurring in patients treated for metastatic breast adenocarcinoma. CASE REPORTS: Case 1: a 72-year-old woman with breast carcinoma relapsing after surgery, chemotherapy, hormone therapy, and without treatment for 6 months, was admitted for an acute erythematous slightly squamous and photo-distributed eruption. On clinical examination, she was found to be presenting polyadenopathy and pleural effusion. Case 2: a 46-year-old woman with a history of breast carcinoma was admitted for an erythematopapular, annular and photo-distributed eruption occurring after a second breast cancer relapse and five months after initiation of docetaxel. A new line of chemotherapy initially resulted in regression of the lesions, and progression of the breast cancer was associated with cutaneous relapse. DISCUSSION: The diagnosis of subacute cutaneous lupus erythematosus was supported in our two patients by the presence of an annular photo-distributed eruption associated with positive testing for anti-Ro/SSA antibodies. Occurrence of the eruption in both cases with relapse of the neoplasia and its improvement after oncological treatment reinforced the diagnosis of paraneoplastic syndrome in one case, and the use of chemotherapy known to trigger lupus could have suggested a diagnosis of drug-induced subacute cutaneous lupus erythematosus. Thus, the association between lupus and cancer is relevant.

[Neonatal septicemias: biological diagnosis and antibiotherapy. Apropos of a series of 46 cases]. / Septicémies néonatales: diagnostic biologique et antibiothérapie. A propos d'une série de 46 cas.

The records of 46 neonates with proven septicaemia were retrospectively studied. Patients could be divided in 2 groups: in group 1 (21 infants) a positive blood culture was obtained before one day of life; in group 2 (25 infants) a positive blood culture was obtained between days 1 and 28. The sensitivity of 9 chemical and bacteriological tests and the efficacy of the initial antibiotic treatment were examined in both groups. Of the 21 germs isolated in patients from group 1, 86% were Gram positive bacteria and 95% were susceptible to ampicillin. Of the 26 germs isolated in patients from group 2, 80% were Gram negative enteric bacteria and 86% were susceptible to cefotaxime. Bacterial tests (gastric aspiration, antigen detection, feces culture) had a better sensibility than biochemical tests (C reactive protein, orosomucoid, fibrinogen).

[Use of imipenem-cilastatin in neonatal septicemias caused by gram-negative bacilli multiresistant to beta-lactam antibiotics]. / Utilisation de l'imipénème-cilastatine dans les septicémies du nouveau-né à bacilles Gram négatif multirésistants aux bêtalactamines.

Seven neonates with septicemia due to Gram negative bacteria resistant to beta-lactam received imipenem-cilastatin therapy. Bacteria isolated were Enterobacter cloacae [3], Enterobacter aerogenes [1], Klebsiella pneumoniae [1], Serratia marcescens [1], Pseudomonas fluorescens [1]. The MICs of imipenem were lower 1 microgram/ml. In 3 children septicemia occurred during previous antimicrobial chemotherapy. 3 IV 60 mg/kg doses of imipenem with amikacin (15 mg kg/d) were administered every day. For five children blood cultures were negative after 48 hours of treatment. E. aerogenes septicemia required pefloxacin because blood cultures remained positive (d5) despite an increased dosage (90 mg/kg/d). All children were cured and imipenem-cilastatin was not responsible for any complication. Those results demonstrate the efficacy of imipenem in the treatment of septicemia in newborns due to multiresistant Gram negative bacteria.