This 14-month-old girl was brought to the emergency room by her parents after a 3-week history of total body swelling and a 2-week history of a rash that began in the diaper area and then spread diffusely. Her hair had become lighter at the roots, and her skin color had become more pallid. She had been breast-fed until age 8 months. Attempts to change her to formula feeds were unsuccessful, since she did not like them. She was started on a diet of a rice-based beverage (Rice Dream, Imagine Foods, Garden City, NY), vegetables, and some meat. Solid-food consumption decreased in the weeks before presentation, as the patient began teething. Kwashiorkor is a disease caused by protein-energy malnutrition. The condition is characterized by a “flaky paint” appearance of the skin, pigment loss, anasarca, and apathy. The term “kwashiorkor,” first used in the English literature in a report by C.D. Williams in The Lancet in 1935, derives from the local word for the disease in Ghana, where it was associated with a maize diet. Still endemic in Africa and in developing countries, kwashiorkor in developed countries has usually been associated with cystic fibrosis or other chronic malabsorptive conditions.
Recently, however, new causes of kwashiorkor in the United States have been reported. Fad diets, perceived and true milk allergy, and nutritional ignorance have been implicated in 14 children diagnosed with kwashiorkor in the United States in the past decade. Rice-based beverages – often popularly referred to as “rice milk,” and containing less than half the amount of protein of breast milk – were a significant part of the feeding regimen in two of these children. Other feeding regimens in these children included brown rice emulsion, goat’s milk, and atole (a liquid emulsion of barley, water, and sugar popular in Mexico). Only two of the cases were associated with poverty. Half of the cases were associated with a perceived or presumed food allergy.
Like the patient presented here, six of these children have had zinc deficiency as well as kwashiorkor. The presence of anasarca and hypoalbuminemia suggested, however, that kwashiorkor was the primary problem. Four patients received zinc supplementation, while the other two were re-fed without zinc supplementation. All six patients recovered. Zinc levels in suspected kwashiorkor patients, however, should be checked; low levels of alkaline phosphatase, a zinc-dependent enzyme, often indicate zinc deficiency.
Other entities in the differential diagnosis of kwashiorkor include free fatty acid or multiple carboxylase deficiency, immunodeficiency syndromes, malabsorption syndromes, cystic fibrosis, and Langerhans cell histiocytosis.
References:
1. Buno IJ, Morelli JG, Weston WL. The Enamel Paint Sign in the Dermatologic Diagnosis of Early-Onset Kwashiorkor. Arch Dermatol. 1998;134:107-8.
2. Carvalho NF, Kenney RD, Carrington PH, Hall DE. Severe Nutritional Deficiencies in Toddlers Resulting From Health Food Milk Alternatives. Pediatrics. 2001;107:e46.
3. Liu T, Frieden IJ. Rice Dream Nondairy Beverages. Arch Dermatol. 2002;138:838.
4. Liu T, Howard RM, Mancini AJ, et al. Kwashiorkor in the United States: Fad Diets, Perceived and True Milk Allergy, and Nutritional Ignorance. Arch Dermatol. 2001;137:630-6.
Description
diffuse brown and erythematous reticulated scaly plaques with a flaky paint appearance
