Long-term Observation of a Case of Bullous Congenital Ichthyosiform Erythroderma (BCIE)

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  • TAKAGI Mami
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences
  • KOBAYASHI Keiko
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences
  • MORITA Akimichi
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences
  • NUMATA Tokio
    Numata Dermatological Clinic

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Other Title
  • 水疱型先天性魚鱗癬様紅皮症の長期観察例
  • 症例 水疱型先天性魚鱗癬様紅皮症の長期観察例
  • ショウレイ スイホウガタ センテンセイ ギョリンセンヨウコウ ヒショウ ノ チョウキ カンサツレイ

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We reported a 12-year-old girl with bullous congenital ichthyosiform erythroderma (BCIE). She had diffuse erythema with membranous scales and erosions 10 cm in diameter over her entire body. The lesions were observed at birth and were gradually desquamated. At the age of 3, washboard-shaped thick scales and bulla began to appear. At the age of 5, she started to take etretinate (0.2-0.5mg/kg/day). Histology at the age of 12 years showed hyperkeratosis and granular degeneration in the epidermis. From those findings, we assigned the diagnosis of BCIE. Etretinate was effective in reducing the thick scaly lesions. The symptoms of desquamated and keratotic plaques gradually decreased. Although her skin was tight, neither bulla nor severe erythema was observed on her body at the age of 20.

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