We are grateful to Dr Schmidt for his comments on our article.1 We fully agree that essential fatty acid deficiency can lead to red, scaly eruptions of the skin.2,3 However, these eruptions do not include ichthyosis. When the diet is supplemented completely, the skin will become normal. When the diet is not well-balanced, several nutritional deficiencies, including essential fatty acid deficiency, can occur in cystic fibrosis. Most common skin findings in patients with cystic fibrosis are an elevated, papular rash, including acrodermatitis enteropathica-like dermatitis; erythema nodosum; rheumatoid nodules; urticaria; purpura; and vasculitis.4 The rash in acrodermatitis enteropathica is characterized by acral and periorificial eczematous or psoriasiform skin eruptions. Ichthyosis has never been reported in cystic fibrosis.The patient we described is extremely small for his age but his weight is normal for his length. Exocrine pancreatic function is completely under control through substitution with pancreatic enzymes and
Oranje AP, Goeteyn M, Vuzevski VD, de Groot R, van Suijlekom-Smit LWA. Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia-Reply. Arch Dermatol. 1991;127(9):1418. doi:10.1001/archderm.1991.01680080158026
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