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Neonatal Pemphigus Foliaceus

2012; Elsevier BV; Volume: 161; Issue: 4 Linguagem: Inglês

10.1016/j.jpeds.2012.04.036

1097-6833

Ana Isabel Lorente Lavirgen, José Bernabeu‐Wittel, J.J. Domínguez-Cruz, Julián Conejo‐Mir,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

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.Figure 2Newborn with flaccid bullae and denuded skin areas on her extremities.View Large Image Figure ViewerDownload Hi-res image Download (PPT)NPF is a transitory autoimmune blistering disease presumed to be caused by the passive transfer of circulating maternal autoantibodies across the placenta.1James K.A. Culton D.A. Diaz L.A. Diagnosis and clinical features of pemphigus foliaceus.Dermatol Clin. 2011; 29: 405-412Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar These autoantibodies are direct against a cell-adhesion glycoprotein (desmoglein 1, 160Kd), which is mainly expressed in the granular layer of the epidermis.1James K.A. Culton D.A. Diaz L.A. Diagnosis and clinical features of pemphigus foliaceus.Dermatol Clin. 2011; 29: 405-412Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar Most infant born of mothers with PF will not develop the disease2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar; only 3 previous cases have been described.2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar, 3Hirsch R. Anderson J. Weinberg J.M. Burnstein P. Echt A. Fermin J. et al.Neonatal pemphigus foliaceus.J Am Acad Dermatol. 2003; 49: S187-S189Abstract Full Text Full Text PDF PubMed Scopus (39) Google Scholar, 4Walker D.C. Kolar K.A. Hebert A.A. Jordon R.E. Neonatal pemphigus foliaceus.Arch Dermatol. 1995; 131: 1308-1310Crossref PubMed Scopus (55) Google Scholar It is believed that the placenta may act as an "in vivo immunoadsorbent" autoantibody.2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google ScholarWe report a new case of neonatal PF established by clinical history and examination. We strongly believe that further tests were not necessary to confirm the diagnosis of NPF because they could have increased the risk of iatrogenia in a neonate, not changing the clinical management, and not improving the diagnoses. 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.1James K.A. Culton D.A. Diaz L.A. Diagnosis and clinical features of pemphigus foliaceus.Dermatol Clin. 2011; 29: 405-412Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar These autoantibodies are direct against a cell-adhesion glycoprotein (desmoglein 1, 160Kd), which is mainly expressed in the granular layer of the epidermis.1James K.A. Culton D.A. Diaz L.A. Diagnosis and clinical features of pemphigus foliaceus.Dermatol Clin. 2011; 29: 405-412Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar Most infant born of mothers with PF will not develop the disease2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar; only 3 previous cases have been described.2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar, 3Hirsch R. Anderson J. Weinberg J.M. Burnstein P. Echt A. Fermin J. et al.Neonatal pemphigus foliaceus.J Am Acad Dermatol. 2003; 49: S187-S189Abstract Full Text Full Text PDF PubMed Scopus (39) Google Scholar, 4Walker D.C. Kolar K.A. Hebert A.A. Jordon R.E. Neonatal pemphigus foliaceus.Arch Dermatol. 1995; 131: 1308-1310Crossref PubMed Scopus (55) Google Scholar It is believed that the placenta may act as an "in vivo immunoadsorbent" autoantibody.2Avalos-Diaz E. Olague-Marchan M. López-Swidersk A. Herrera-Esparza R. Díaz L.A. Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.J Am Acad Dermatol. 2000; 43: 1130-1134Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar We report a new case of neonatal PF established by clinical history and examination. We strongly believe that further tests were not necessary to confirm the diagnosis of NPF because they could have increased the risk of iatrogenia in a neonate, not changing the clinical management, and not improving the diagnoses.