Neonatal pemphigus foliaceus
Autor: | José Bernabeu-Wittel, Ana Isabel Lorente Lavirgen, Julián Conejo-Mir, Javier Domínguez-Cruz |
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Rok vydání: | 2012 |
Předmět: |
Adult
medicine.medical_specialty Physical examination Azathioprine chemistry.chemical_compound Prednisone Pregnancy Medicine Humans Maternal-Fetal Exchange Pemphigus foliaceus integumentary system medicine.diagnostic_test business.industry Infant Newborn medicine.disease Betamethasone valerate Dermatology Pregnancy Complications chemistry Desmoglein 1 Pediatrics Perinatology and Child Health Prednisolone Female business Pemphigus medicine.drug |
Zdroj: | The Journal of pediatrics. 161(4) |
ISSN: | 1097-6833 |
Popis: | Figure 2. Newborn with flaccid bullae and denuded skin areas on her extremities. A 1-hour-old female patient was referred with skin erosions on her extremities. The patient’s mother was a 33-year-old, otherwise-healthy woman with a 6-year history of pemphigus foliaceus (PF). The patient’s mother had been treated with different immunosuppressive medications, including topical steroids ointments, intermittent oral prednisone, azathioprine, and intermittent intravenous treatment with immunoglobulins. Three months before becoming pregnant, she discontinued all treatments, and a cutaneous relapse of PF occurred at the sixth month of pregnancy, for which she received topical and oral steroids (prednisone 0.5 mg/kg/daily) until delivery. At the time of delivery, the mother had multiple superficial erosions and crusted skin patches on her scalp, face, and trunk (Figure 1), and the infant had flaccid bullae and denuded skin areas on her extremities (Figure 2). Given the clinical presentation and the mother’s pre-existing disease, we diagnosed the infant with neonatal pemphigus foliaceus (NPF). Carefully, we initiated wound care, oral steroids (prednisolone 0.5 mg/kg/daily for a week), and prescribed betamethasone valerate 0.1% ointment. At 4 weeks of age, the clinical examination of her skin was normal. NPF is a transitory autoimmune blistering disease presumed to be caused by the passive transfer of circulating maternal autoantibodies across the placenta. These autoantibodies are direct against a cell-adhesion glycoprotein (desmoglein 1, 160Kd), which is mainly expressed in the granular layer of the epidermis. Most infant born of mothers with PF |
Databáze: | OpenAIRE |
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