OBJECTIVETo acquire more knowledge about neonatal lupus erythematosus (NLE).

METHODSeven cases with neonatal lupus erythematosus who were seen in this hospital from 1990 to 2009 are reported in this paper and 87 cases reported previously from 1980 to now in China were reviewed. The clinical manifestations, serum autoantibodies, treatment and results of long-term follow-up are analyzed and summarized.

RESULTTotally 94 cases were summarized. Male/female ratio was 48/46; 73 cases had skin rash; 23 had heart abnormality, among whom 13 had cardiac conductive problems including 8 cases of atrioventricular blockage (AVB) (3 degree I, 3 degree II and 2 degree III) and 5 cases of right bundle branch block cases (RBBB). Nine cases had anatomical abnormality including 5 cases of atrial septal defect (ASD), 2 cases of ventricular septal defect (VSD) and 2 cases of atrial enlargements. Forty-four cases had hematological problems including 28 with thrombocytopenia, 11 with leukocytopenia and 34 with anemia. Thirty cases had hepatic abnormality, including 24 liver dysfunction, 22 hepatomegaly, 6 splenomegaly and 3 cholestasis. Three cases had nephropathy; 3 had elevated creatine kinase; 2 had nervous disorder. Among the 94 cases, 86 (91.5%) were positive for anti-SSA, 51 (54.3%) anti-SSB, 16 anti-ds-DNA, 14 anti-U1-RNP, 13 anti-Sm (+), 6 anti-RNP and 4 anti-rRNP(+). Among the corresponding mothers, 39 cases (44.8%) were asymptomatic before pregnancy, 35 had SLE, 5 had SCLE, 3 had Sjogren syndrome, 2 had chilblain, photosensitivity and arthralgia, respectively, 1 had rheumatoid arthritis and 1 had psoriasis. During pregnancy, 8 mothers developed SLE. Totally 48 mothers (51.1%) suffered from LE. Together with 15 mothers who had transient skin rash during the pregnancy, there were 23 mothers (59%) who had new clinical manifestation among the 39 asymptomatic mothers. Twenty NLE cases accepted glucocorticoid treatment, 4 of them were treated with intravenous immunoglobulin. Sixty-eight cases were followed up for up to 12 years, 58 cases were healthy, 5 cases improved and 3 died. Two cases still had grade III AVB without pacemaker.

CONCLUSIONNLE is a rare acquired autoimmune disease. Although nearly half of the mothers were asymptomatic before pregnancy, more than half of them developed LE or other symptoms. The clinical presentations in Chinese cases include a transient rash, cardiac lesion while grade III AVB was rare, hematological changes and liver impairments which were common but not serious. Anti-SSA and/or anti-SSB were the most related autoantibody. Most patients with NLE have relatively good prognosis.

Adult ; Autoantibodies ; blood ; Female ; Follow-Up Studies ; Humans ; Infant, Newborn ; Lupus Erythematosus, Cutaneous ; drug therapy ; pathology ; Lupus Erythematosus, Systemic ; congenital ; diagnosis ; drug therapy ; pathology ; Male ; Mothers ; Pregnancy ; Prognosis ; Retrospective Studies

OBJECTIVENeonatal lupus erythematosus (NLE) is an uncommon autoimmune disease passively transmitted from the mother in which there is transplacental passage of maternal antibodies. It is often misdiagnosed as intrauterine infection or sepsis. The main purpose of this retrospective study was to improve the understanding of pathogenesis and clinical manifestations of NLE.

METHODSClinical manifestations, results of the tests for antinuclear antibodies (ANA), anti-Ro/SSA, anti-La/SSB and anti-dsDNA antibodies in both infants with NLE (8 cases) and their mothers and head ultrasound and CT scans of the infants were analyzed. Follow-up was performed until one and ahalf years of age or all the abnormalities had been resolved.

RESULTSTotally 8 cases (3 males and 5 females) matched the criteria for diagnosis of NLE from September 2003 to February 2006, among whom 4 were small for gestational age and one was born prematurely. Mean gestational age was 38.1 +/- 1.9 weeks, mean birth weight 2 605 +/- 420 grams, mean admission age 22.4 +/- 27.7 days (2 hours-72 days) and mean age of onset 9.4 +/- 12.1 days (0 - 28 days). The common clinical manifestations included cutaneous lupus lesions (8 infants), neural system abnormalities (2 infants) and congenital heart block (2 infants). The skin of the infants exhibited annular, erythematous or desquamative lesions. They all disappeared before 6 months of age. One patient presented with grade III atrioventricular block and was delivered by cesarean section because of "fetal distress". He did not recover at one and a half years follow-up. One infant was hypotonic with delayed neuro-motor development initially and during follow-up with both abnormal neonatal behavioral neurological assessment (NBNA) and image findings. CT showed generalized low density involving periventricular area and deep white matter at one week of age. At the age of one and a half years, he presented with normal mental development index determined by CDCC infant intelligence mensuration. Other abnormal clinical findings included hepatosplenomegaly, anemia, thrombocytopenia, cholestasis and elevated liver enzymes, which were all resolved before 6 months of age. Only 3 mothers of the NLE infants were diagnosed as lupus erythematosus before parturition and only one received partial therapy. At least anti-Ro/SSA antibody or anti-La/SSB antibody or ANA was found in the affected patients. Seven cases had circulating anti-Ro and/or anti-La antibodies in the mothers and in the newborns, while ANA was positive in 7 newborns and in all mothers. All the clinical symptoms except congenital heart block disappeared before 18 months of age. No special intervention was applied.

CONCLUSIONSerum auto-antibodies should be investigated to rule out NLE when there is congenital heart block or rashes or thrombocytopenia presented in a neonate, despite there is no maternal history. Central nervous system abnormalities in NLE are likely to be transient and whether it will cause long term sequelae is uncertain.

Autoimmune Diseases ; pathology ; Female ; Humans ; Infant ; Infant, Newborn ; Infant, Newborn, Diseases ; diagnosis ; Lupus Erythematosus, Cutaneous ; diagnosis ; pathology ; physiopathology ; Male ; Retrospective Studies

Antibodies, Antinuclear ; blood ; Female ; Follow-Up Studies ; Heart Block ; congenital ; Humans ; Infant ; Infant, Newborn ; Lupus Erythematosus, Cutaneous ; complications ; drug therapy ; pathology ; Male

Tinea incognito (TI) is a dermatophytic infection which has lost its typical clinical appearance because of improper use of steroids or calcineurin inhibitors. The incidence of TI is increasing nowadays. We conducted retrospective review on 283 patients with TI from 25 dermatology training hospitals in Korea from 2002-2010 to investigate the demographical, clinical, and mycological characteristics of TI, and to determine the associated risk factors. More than half (59.3%) patients were previously treated by non-dermatologists or self-treated. The mean duration of TI was 15.0 +/- 25.3 months. The most common clinical manifestations were eczema-like lesion, psoriasis-like, and lupus erythematosus-like lesion. The trunk and face were frequently involved, and 91 patients (32.2%) also had coexisting fungal infections. Among 67 isolated strains, Trichophyton rubrum was the most frequently detected (73.1%). This is the largest study of TI reported to date and the first investigational report concerning TI in Korea. We suggest that doctors should consider TI when a patient has intractable eczema-like lesions accompanied by tinea pedis/unguium. Furthermore, there should be a policy change, which would make over-the-counter high-potency topical steroids less accessible in some countries, including Korea.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Demography ; Eczema/pathology ; Face/pathology ; Female ; Humans ; Lupus Erythematosus, Cutaneous/pathology ; Male ; Middle Aged ; Psoriasis/pathology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Tinea/*diagnosis/microbiology ; Trichophyton/isolation & purification ; Young Adult