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Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia-Reply
Arnold P. Oranje, MD, PhD;
Marleen Goeteyn, MD;
Vojislav D. Vuzevski, MD;
Ronald de Groot, MD;
Lisette W. A. van Suijlekom-Smit, MD
Department of Dermatovenereology University Hospital Rotterdam PO Box 70029 3000 LL Rotterdam, the Netherlands; Department of Dermatology Ghent University Hospital De Pintelaan 185 9000 Ghent, Belgium; Department of Pathological Anatomy Erasmus University Dr Molewaterplein 40 3000 DR Rotterdam, the Netherlands; Department of Pediatrics University Hospital Rotterdam/ Sophia Children's Hospital PO Box 70029 3000 LL Rotterdam, the Netherlands.
Arch Dermatol. 1991;127(9):1418.
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In Reply.—
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
. . . [Full Text PDF of this Article]
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