Twins with neonatal pemphigus vulgaris born to a mother with pemphigus vulgaris: a case report.

Dichorionic diamniotic twins were born at 37 weeks of gestation by cesarean section to a 34-year-old primigravid Japanese woman because the first twin was in breech presentation. The mother had been diagnosed with pemphigus vulgaris prior to her pregnancy. In addition to a high antidesmoglein 3 autoantibody titer, flaccid bullae and erosions on both of the twins' lips and in their oral cavities at 13 days of age led to the diagnosis of neonatal pemphigus vulgaris. This case highlights the need for awareness that pemphigus vulgaris may not occur immediately after birth.

Pregnancy-triggered maternal pemphigus vulgaris with persistent gingival lesions.

Pregnancy as a triggering factor of pemphigus vulgaris (PV) seems to be quite a rare phenomenon. According to a recent review, only 38 reports describing 49 pregnant women with PV have been published in English language literature. A 34-year-old woman is described with pregnancy-triggered PV showing persistent gingival erosions. In addition, a shift from mucocutaneous to mucosal-dominant clinical variant of the disease in the mother, suggested by clinical features, was confirmed at the molecular level by determination of anti-desmoglein (DSG) 1 and anti-DSG 3 circulating IgG autoantibodies with ELISA. The case presented shows that PV in pregnancy requires care by a gynecologist, dermatologist and neonatologist. They all should be aware of the peculiarities of PV in pregnancy and be willing to cooperate with each other. Noteworhily, in contrast to cutaneous lesions, gingival lesions seen in the mother in the mucosal-dominant stage of her PV after delivery were unresponsive to intravenous and oral corticosteroid/oral cyclophosphamide treatment scheme.

Cleavage isn't everything: potential novel mechanisms of exfoliative toxin-mediated blistering.

This Commentary describes breakthroughs in understanding the interactions between desmoglein 1 and plakogloben in staphylococcal-mediated blistering skin diseases.

Neonatal pemphigus vulgaris in an infant born to a mother with pemphigus vulgaris in remission.

Neonatal pemphigus vulgaris is a rare autoimmune disease that is caused by transplacental passage of pemphigus vulgaris autoantibodies. The association of maternal pemphigus vulgaris with neonatal disease pemphigus vulgaris has been only rarely reported. We describe an infant with pemphigus vulgaris born to a mother whose disease was in remission.

Pemphigus, pregnancy, and plasmapheresis.

Pemphigus vulgaris (PV) is an autoimmune blistering disorder that usually occurs in the fifth and sixth decades of life but may occur at younger ages and during pregnancy. Circulating intercellular antibodies directed at desmosomal proteins may cross the placenta and place children at risk for neonatal pemphigus (NP). We describe the case of a pregnant woman with PV treated successfully with a combination of systemic corticosteroids and plasmapheresis. The possibility of PV should be considered in any pregnant woman with a worsening, widespread, mucocutaneous, blistering disease. Plasmapheresis offers a useful alternative to immunosuppressive therapy in the setting of pregnancy.

Neonatal pemphigus foliaceus.

The term "pemphigus" refers to a group of diseases that are characterized by the presence of cutaneous or mucosal blisters and erosions, and antiepidermal autoantibodies. There are several case reports of neonatal pemphigus vulgaris in the literature. Although pemphigus foliaceus antibodies have been shown to cross the placenta, to our knowledge, this is only the second reported case of neonatal pemphigus foliaceus. The proposed mechanism of disease transfer is the passive transfer of maternal IgG antibodies across the placenta.

Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns.

We report a case of neonatal pemphigus vulgaris presenting with skin lesions on the head, genital area, and right foot. Pemphigus vulgaris was diagnosed by the presence of circulating autoantibodies predominantly of the IgG4 subtype by indirect immunofluorescence microscopy and by enzyme-linked immunosorbent assay using recombinant desmoglein 3. This case demonstrates the pathogenic relevance of IgG4 autoantibodies to desmoglein 3 in the skin of neonates.

Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris.

The clinical phenotype of pemphigus is well explained by the combination of desmoglein (Dsg) 1 and Dsg3 distribution pattern and antiDsg autoantibody profile (Dsg compensation theory). It has been reported that neonatal skin has a similar Dsg distribution pattern to adult mucosal epithelia. We describe a newborn girl with mucocutaneous pemphigus vulgaris (PV) from a mother with mucosal dominant PV. The mother had had painful oral erosions for at least 7 months. Histopathological examination and direct and indirect immunofluorescence studies confirmed the diagnosis of PV and neonatal PV in the mother and daughter, respectively. The mother had a high titre of anti-Dsg3 IgG and a low titre of antiDsg1 IgG, while the neonate had only a high titre of anti-Dsg3 IgG, but no detectable antiDsg1 IgG. AntiDsg3 IgG, which caused the oral dominant phenotype in the mother, induced extensive oral as well as cutaneous lesions in the neonate. Our case provides clinical evidence for the Dsg compensation theory in neonatal PV.

Neonatal pemphigus vulgaris associated with mild oral pemphigus vulgaris in the mother during pregnancy.

We report a neonate with immunofluorescence-proven pemphigus vulgaris. The condition presented at birth with widespread skin erosions and ulceration of the oral mucosa. Histopathological and immunofluorescence studies confirmed pemphigus vulgaris. The mother had mild oral pemphigus vulgaris treated during pregnancy with topical corticosteroids. All the neonate's skin erosions had crust formation at day 2 but healed completely within 2 weeks.

Pemphigus vulgaris in pregnancy with favourable foetal prognosis.

Pemphigus vulgaris is an immunobullous disease affecting the skin and mucous membranes most commonly during the fifth and sixth decades of life. Its occurrence in pregnancy is rare. We now report two severe cases of the disorder presenting during pregnancy and discuss its potential effects on the foetus and its management in pregnancy.

Neonatal pemphigus vulgaris in a child born to a woman in remission.

We describe the tenth reported case of neonatal pemphigus that mimicked Bart's syndrome and review previously published cases. Unlike previous cases, the child was born with significant blistering to a mother who was in complete remission throughout the pregnancy. High antepartum maternal titers of anti-intercellular space antibodies, increased maternal disease activity, and maternal disease that requires high doses of corticosteroids or use of combined therapy correlate with poor fetal outcome, including intrauterine death.

Neonatal pemphigus vulgaris: transplacental transmission of antibodies.

A male infant with skin lesions was born to a 28-year-old mother who was under treatment for pemphigus vulgaris (PV), diagnosed eight years earlier. Circulating IgG class pemphigus antibody was found in the infant's blood, and deposition of IgG in the intercellular spaces of the epidermis was seen. The infant's lesions resolved within three weeks, and pemphigus antibody titer became negative by seven weeks. The pathogenetic role of PV antibodies and the risk for a fetus of a mother suffering from PV are discussed.

Neonatal pemphigus vulgaris: role of passively transferred pemphigus antibodies.

A neonate with oral lesions of pemphigus vulgaris is described. The diagnosis was confirmed by cytological examination of lesions, positive intercellular IgG staining of the acantholytic cells, and presence of pemphigus antibodies in his serum. At the time of delivery, the child's mother had active pemphigus vulgaris, and it is suggested that this was a case of passively transferred disease.