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Quiz

Clinical Challenge

Scaly Dermatitis and Edema in an Irritable Child

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA

Published Online: January 7, 2021

JAMA Clinical Challenge

Article Information and Disclosures

Markus D. Boos, MD, PhD¹;

Shanelle Briggs, BA, BS²

Author Affiliations

¹Division of Dermatology, Department of Pediatrics, University of Washington School of Medicine, Seattle Children’s Hospital, Seattle
²University of Washington School of Medicine, Seattle

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Read Case Take Quiz Answer

A 19-month-old boy presented for evaluation of widespread edema and acute dermatitis. Following an upper respiratory tract infection 1 month prior, he developed disseminated red, scaly patches and a progressive eruption of hyperpigmented and hypopigmented patches of skin. He had occasional nonbloody loose stools but no fevers or vomiting. Although he was otherwise well, his growth had fallen from the 75th to the 25th percentile for height and weight over the preceding 9 months, and he could speak only 2 words. His medical history was notable for atopic dermatitis and diarrhea in infancy that prompted allergy testing at age 6 months; he was subsequently diagnosed with milk, egg, and peanut allergies. His parents avoided feeding him these foods, providing him only soy-based milk and formula, with improvement of dermatitis, until age 1 year. Since then his diet had consisted of multiple rice milk feeds daily, supplemented only with pureed fruits and vegetables, pasta, snack puffs, and sunflower butter. The patient had no history of meconium ileus or family history of cystic fibrosis. There was no recent travel and no changes to the home environment.

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Kwashiorkor

A. Check levels of protein, albumin, prealbumin, and other nutritional laboratory measures

The key to the correct diagnosis in this case is recognizing edema, exfoliative dermatitis, and pigmentary dilution in an irritable child who consumes a relatively protein-deficient diet. This can be confirmed via laboratory evaluation revealing low levels of prealbumin, albumin, and total protein, as identified in this patient (prealbumin, 11 mg/dL; albumin, 1.7 g/dL; total protein, 3.8 g/dL) (choice A). Obtaining a urinalysis (choice B) is incorrect, as the edema of kwashiorkor is not secondary to protein loss in urine. The histologic features of kwashiorkor are not unique, and biopsy is not required for diagnosis (choice C).¹^- 3 Kwashiorkor may be mistaken for atopic dermatitis or precipitated by well-intentioned parents who restrict their child’s nutrition in an effort to treat the condition.⁴ While topical steroids (choice D) may improve the dermatitis of kwashiorkor, this would not address the underlying cause.

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Article Information

Corresponding Author: Markus Boos, MD, PhD; Seattle Children’s Hospital, 4800 Sand Point Way NE, OC.9.833, Seattle, WA 98105 (markus.boos@seattlechildrens.org).

Published Online: January 7, 2021. doi:10.1001/jama.2020.10429

Conflict of Interest Disclosures: None reported.

Additional Information: We thank the patient’s mother for providing permission to share the patient’s information.

References

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(5):630-636.PubMed Google Scholar

Guidelines for the Inpatient Treatment of Severely Malnourished Children. World Health Organization. Published 2003. Accessed July 22, 2020. https://www.who.int/nutrition/publications/guide_inpatient_text.pdf

Brewster DR . Critical appraisal of the management of severe malnutrition, 2: dietary management. J Paediatr Child Health. 2006;42(10):575-582. doi:10.1111/j.1440-1754.2006.00932.x PubMed Google Scholar Crossref

Henrique de S B Xavier M , De Magalhães E , Ferraz Oliveira G , Keltke Magalhães M , Prates de Almeida E Oliveira C , Bragança Oliveira N . A child with kwashiorkor misdiagnosed as atopic dermatitis. Dermatol Online J. 2017;23(5):23(5):13030/qt4dd7h96r.PubMed Google Scholar

Grover Z , Ee LC . Protein energy malnutrition. Pediatr Clin North Am. 2009;56(5):1055-1068. doi:10.1016/j.pcl.2009.07.001 PubMed Google Scholar Crossref

Katz KA , Mahlberg MJ , Honig PJ , Yan AC . Rice nightmare: kwashiorkor in 2 Philadelphia-area infants fed Rice Dream beverage [published correction appears in J Am Acad Dermatol. 2005;53(3):496]. J Am Acad Dermatol. 2005;52(5)(suppl 1):S69-S72. doi:10.1016/j.jaad.2004.07.056 PubMed Google Scholar Crossref

Sandy NS , Nogueira RJN . Nutritional treatment of a young infant with cystic fibrosis presenting with severe kwashiorkor dermatosis. J Trop Pediatr. 2019;65(6):634-637. doi:10.1093/tropej/fmz008 PubMed Google Scholar Crossref

Tierney EP , Sage RJ , Shwayder T . Kwashiorkor from a severe dietary restriction in an 8-month infant in suburban Detroit, Michigan: case report and review of the literature. Int J Dermatol. 2010;49(5):500-506. doi:10.1111/j.1365-4632.2010.04253.x PubMed Google Scholar Crossref

Downie ML , Gallibois C , Parekh RS , Noone DG . Nephrotic syndrome in infants and children: pathophysiology and management. Paediatr Int Child Health. 2017;37(4):248-258. doi:10.1080/20469047.2017.1374003 PubMed Google Scholar Crossref

Brewster DR . Critical appraisal of the management of severe malnutrition, 3: complications. J Paediatr Child Health. 2006;42(10):583-593. doi:10.1111/j.1440-1754.2006.00933.x PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.