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April 1973


Arch Dermatol. 1973;107(4):619-621. doi:10.1001/archderm.1973.01620190087024

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Bullous Congenital Ichthyosiform Erythroderma. Presented by George Ramelli, MD (by invitation).  A 4-year-old white girl developed a generalized bullous eruption approximately 12 hours after birth. During the first year of life, she had exacerbations and remissions of an erythematous, bullous eruption. She then developed extensive hyperkeratosis, mainly of the flexural areas, and scaling of the scalp. The patient then had no bullae for two years. Several large bullous lesions subsequently appeared in December 1971. There is no family history of a similar problem. Treatment consisted of 10% urea cream, with moderate improvement.

Discussion  Dr. Leo Indianer: My diagnosis is epidermolytic hyperkeratosis, compatible with the clinical entity of congenital ichthyosiform eruption.Dr. William B. Reed: Epidermolytic hyperkeratosis is a histopathological term applicable to many disorders besides congenital ichthyosiform erythroderma. E. A. Cockayne classified the bullous and nonbullous forms of erythroderma. This type is autosomal dominant. The condition has a bad prognosis;

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