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September 1991

Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia

Author Affiliations

Department of Dermatology The Johns Hopkins Medical Institutions 600 N Wolfe St Baltimore, MD 21205

Arch Dermatol. 1991;127(9):1417-1418. doi:10.1001/archderm.1991.01680080157025

To the Editor.—  Recently, Goeteyn and coworkers1 published a case report on the Schwachman syndrome and suggested that skin findings are frequently a component of this syndrome.What caught my eye in this article were the statements that essential fatty acid (EFA) deficiency and red scaly eruptions have not been reported in cystic fibrosis. In fact, there are several publications describing each of these in patients with cystic fibrosis.2-4 In addition, my own experience suggests this is not a rare occurrence.5It is thought that the pancreatic exocrine dysfunction seen in cystic fibrosis causes multiple nutritional deficiencies.2 These include decreased serum zinc, protein, and EFA concentrations. Each deficiency can cause a red scaly eruption. Dietary supplementation, including pancreatic enzymes, results in rapid resolution of the dermatitis.The patient described by Goeteyn et al had pancreatic dysfunction similar to that found in cystic fibrosis. He also

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