Neonatal pemphigus foliaceus

Autor: José Bernabeu-Wittel, Ana Isabel Lorente Lavirgen, Julián Conejo-Mir, Javier Domínguez-Cruz
Rok vydání: 2012
Předmět:
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
Externí odkaz: