Childhood, neonatal, and stillborn pemphigus vulgaris

Bjarnason, Bolli; Flosadóttir, Ellen; DDS, None

doi:10.1046/j.1365-4362.1999.00764.x

Cited by 52 publications

(58 citation statements)

References 54 publications

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“…Cases of neonatal pemphigus occurring while the mother is in clinical remission or is asymptomatic, such as the florid presentation of our patient in spite of the subtle maternal disease, suggest that neonatal skin is more sensitive to circulating anti-Dsg3 antibodies than maternal skin. 1,3,4 However, there is no clear correlation between the severity and pattern of maternal involvement, level of antibody titers or prematurity, and the neonatal clinical presentation. 5 Of note, in some cases of neonatal pemphigus, 3,4 including our case, DIF is positive for IgG and C3.…”

Section: Discussionmentioning

confidence: 99%

Blisters and Erosions in a Neonate

2014

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A newborn male infant was transferred to the neonatal intensive care unit for widespread vesicles and erosions evident at birth, following a spontaneous vaginal delivery complicated by fetal meconium exposure. The mother, initially unavailable for examination because she remained at the facility where she had given birth, reported via telephone that she was healthy except for a urinary tract infection for which she had taken a 7-day course of nitrofurantoin monohydrate approximately 3 to 4 weeks before delivery. She denied a history of herpesvirus infection, blistering disorder, or autoimmune disease. On physical examination, the neonate exhibited extensive erosions rimmed by purulent vesicles involving the groin, neck, scalp, fingers, and left side of the upper chest ( Figure, A and B). Mucous membranes were spared. Biopsy specimens were obtained, with results as shown in the Figure,

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Section: Discussionmentioning

confidence: 99%

Blisters and Erosions in a Neonate

2014

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“…However, high-dose and prolonged administration of these agents may result in numerous well-known side effects. Adjuvant steroid-sparing therapies, such as azathioprine [5,6,7,8], methotrexate [6,7], oral gold [9], mycophenolate mofetil [10], intravenous immunoglobulins (IVIg) [11] and dapsone [7,12], have been previously reported as effective in isolated cases of CPV. Therefore, evidence-based guidelines have not been reported for CPV yet.…”

Section: Discussionmentioning

confidence: 99%

Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

et al. 2010

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Pemphigus vulgaris is an infrequent but life-threatening autoimmune blistering disease that is rare in the pediatric age. Although the mainstay of therapy for childhood pemphigus vulgaris (CPV) is steroids, adjuvant immunosuppressive drugs are often needed to control the disease. Thus, an important part of CPV morbidity can be related to treatment. We report the youngest CPV patient described in the English literature, an 18-month-old boy, who has been followed up for 16 years. During this period, the patient has received several immunosuppressive therapies with variable response. He has finally achieved a long-lasting and complete remission with rituximab. Successful treatment with rituximab has previously been reported in 6 cases of CPV, in whom rituximab presented a good side effect profile. Our patient has experienced a chronic and severe clinical course with multiple flares eventually developing vegetative lesions. Since he presented refractoriness to multiple therapies, we administered rituximab. The introduction of this drug led to a dramatic clinical response and a long-term clinical remission. Based on the experience of this case and the data reported in the literature, we believe that rituximab may be a safe and efficacious agent for the treatment of severe CPV.

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“…Ähnlich wie bei Erwachsenen wurden auch bei Kindern mit PF intensive Sonneneinwirkung und Medikamenteneinnahme als Triggerfaktoren vermutet, ebenso wie bakterielle und virale Begleitinfektionen. Auch das klinische Bild bei Kindern ähnelt der adulten Form: Charakteristisch wichtig für die differenzialdiagnostische Abgrenzung des Pemphigus vulgaris, der bei Kindern meist eine schlechtere Prognose hat, ist die Aussparung der Schleimhäute [6]. Gesicht, obere Rumpfregion und Arme sind Prä-dilektionsstellen auch des Pemphigus foliaceus bei Kindern und Jugendlichen.…”

Section: Nichtendemischer Pemphigus Foliaceus Bei Kindern Und Jugendlunclassified

Pemphiguserkrankungen bei Kindern und Jugendlichen

Meurer

2009

Hautarzt

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The pemphigus diseases, which include some of the most severe bullous autoimmune skin reactions, are seen predominantly in middle-aged and elderly individuals. Only endemic pemphigus foliaceus in South America most frequently affects juveniles and children. All non-endemic pemphigus diseases, including paraneoplastic pemphigus, have been reported to occur in adolescents and even very rarely in children younger than 10 years. Pemphigus vulgaris in pregnancy represents a frequently overseen medical problem and may result in fetal growth retardation, intrauterine death, premature delivery and - in about 30% - in neonatal pemphigus vulgaris of the newborn. Contrary to pemphigus vulgaris, the transplacental crossing of autoantibodies against desmoglein1 in pregnant women with pemphigus foliaceus hardly ever leads to neonatal skin lesions in the offspring. This phenomenon can be explained by differences in the distribution and cross-compensation of the pemphigus antigens desmoglein3 and 1 in neonatal and adult skin or mucosa, respectively.

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Childhood, neonatal, and stillborn pemphigus vulgaris

Cited by 52 publications

References 54 publications

Blisters and Erosions in a Neonate

Blisters and Erosions in a Neonate

Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

Pemphiguserkrankungen bei Kindern und Jugendlichen

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